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Keywords: porphyria
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Clin Sci (Lond) (1987) 72 (1): 103–112.
Published: 01 January 1987
.... In two cases of acute intermittent porphyria with overwhelming neuropathy the maximum plasma levels of ALA were 9 and 12 μmol/l. Haematin infusion decreased the ALA levels but without obvious clinical benefit. Limited neurological recovery occurred without major reduction in plasma levels of ALA. 8. One...
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Clin Sci Mol Med (1978) 55 (5): 461–469.
Published: 01 November 1978
... resulted in the development of porphyria in rats, which biochemically closely resembles symptomatic porphyria in humans, with elevation of urinary uroporphyrin excretion, hepatic uroporphyrin content, and hepatic cytochrome P -450 content, in addition to appearance of porphyrins of the isocoproporphyrin (P...
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Clin Sci Mol Med (1978) 54 (3): 251–256.
Published: 01 March 1978
...E. G. Astrup 1. Ten subjects with acute intermittent porphyria from three different families, and 92 relatives, were investigated for their erythrocyte uroporphyrinogen I synthase (EC 4.3.1.8) activities by the spectrofluorimetric method described and for their urinary concentrations of δ...
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Clin Sci Mol Med (1977) 53 (2): 111–115.
Published: 01 August 1977
... concentration also rose but to a lesser extent than that in the non-siderotic rats. 4. Hexachlorobenzene-induced porphyria would seem to be attributable to inhibition or inactivation of hepatic uroporphyrinogen decarboxylase. Hepatic siderosis has a synergistic effect with hexachlorobenzene on this enzyme...
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Clin Sci Mol Med (1976) 51 (1): 71–80.
Published: 01 July 1976
...G. H. Elder; J. O. Evans; S. A. Matlin 1. A new method for the measurement of uroporphyrinogen decarboxylase (EC 4.1.1.37) in rat liver homogenates, with 5-carboxyl porphyrinogen as substrate, is described. 2. The administration of a diet containing 0·3% (w/w) hexachlorobenzene produces porphyria...
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Clin Sci Mol Med (1974) 46 (2): 207–222.
Published: 01 February 1974
... porphyria. 2. The seven patients examined in acute attack of this disease had significantly increased excretions of the urinary sulphate and/or glucuronide conjugates of dehydroepiandrosterone (3β-hydroxyandrost-5-en-17-one) and aetiocholanolone (3α-hydroxy-5β-androstan-17-one). There were increases...
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Clin Sci (1972) 43 (2): 299–302.
Published: 01 August 1972
...M. R. Moore; G. G. Thompson; A. Goldberg 1. The levels of ‘X-porphyrin’, a porphyrin-peptide complex, have been studied in the faeces of patients with different types of porphyria, as well as in fifty normal subjects. 2. These levels have been shown to be significantly elevated in untreated...