Literature DB >> 449661

Hematin therapy for acute porphyria.

J M Lamon, B C Frykholm, R A Hess, D P Tschudy.   

Abstract

1. A therapeutic trial of intravenous hematin is presented. Eleven cases of AIP and one of VP who did not improve with conventional treatment (high carbohydrate intake) received this new agent. 2. Urinary ALA, PBG and, when possible, uroporphyrin and coproporphyrin were used to monitor the chemical response to the treatment. Objective clinical parameters of hypertension and tachycardia were followed when present in addition to subjective estimates of acute porphyric symptomatology (abdominal pain, backache, extremity pain and paresthesias, weakness, depression, etc.). 3. At a dosage of approximately 3 mg/kg, diminution of urinary ALA and PBG excretion was achieved in every patients. Hypertension and tachycardia improved in those instances where they were observed in association with the attack. Also, subjective improvements in the clinical status of the patients were observed frequently. 4. Hematin appears to be a promising therapeutic agent for the treatment of acute attack forms of porphyria.

Entities:  

Mesh:

Substances:

Year:  1979        PMID: 449661     DOI: 10.1097/00005792-197905000-00005

Source DB:  PubMed          Journal:  Medicine (Baltimore)        ISSN: 0025-7974            Impact factor:   1.889


  16 in total

Review 1.  Targeting the Nrf2-Heme Oxygenase-1 Axis after Intracerebral Hemorrhage.

Authors:  Jing Chen-Roetling; Raymond F Regan
Journal:  Curr Pharm Des       Date:  2017       Impact factor: 3.116

2.  Differential effects of metalloporphyrins on messenger RNA levels of delta-aminolevulinate synthase and heme oxygenase. Studies in cultured chick embryo liver cells.

Authors:  E E Cable; J A Pepe; N C Karamitsios; R W Lambrecht; H L Bonkovsky
Journal:  J Clin Invest       Date:  1994-08       Impact factor: 14.808

3.  Activation of factor XII-dependent pathways in human plasma by hematin and protoporphyrin.

Authors:  C G Becker; M Wagner; A P Kaplan; M Silverberg; R W Grady; H Liem; U Muller-Eberhard
Journal:  J Clin Invest       Date:  1985-08       Impact factor: 14.808

Review 4.  Management of attacks of acute porphyria.

Authors:  A C Laiwah; K E McColl
Journal:  Drugs       Date:  1987-11       Impact factor: 9.546

Review 5.  The acute hepatic porphyrias.

Authors:  Bruce Wang
Journal:  Transl Gastroenterol Hepatol       Date:  2021-04-05

6.  Hematin therapy in porphyric attacks.

Authors:  C A Pierach; I Bossenmaier; R Cardinal; M Weimer; C J Watson
Journal:  Klin Wochenschr       Date:  1980-08-15

7.  Acute intermittent porphyria--an unusual cause of "surgical" abdomen. Response to propranolol therapy.

Authors:  I Srugo; E Said; S Korman; M Jaffe
Journal:  Eur J Pediatr       Date:  1987-05       Impact factor: 3.183

8.  Incorporation of haemoglobin haem into the rat hepatic haemoproteins tryptophan pyrrolase and cytochrome P-450.

Authors:  J F Wyman; J L Gollan; W Settle; G C Farrell; M A Correia
Journal:  Biochem J       Date:  1986-09-15       Impact factor: 3.857

9.  Immune stimulatory and anti-tumour properties of haemin.

Authors:  A Tsuji; J Wang; K H Stenzel; A Novogrodsky
Journal:  Clin Exp Immunol       Date:  1993-09       Impact factor: 4.330

10.  Prevention of neonatal hyperbilirubinemia by tin protoporphyrin IX, a potent competitive inhibitor of heme oxidation.

Authors:  G S Drummond; A Kappas
Journal:  Proc Natl Acad Sci U S A       Date:  1981-10       Impact factor: 11.205

View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.