.... 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...

...K. E. L. McColl; A. M. Wallace; M. R. Moore; G. G. Thompson; A. Goldberg 1. The timing of onset of attacks of acute intermittent porphyria (AIP) in relation to the menstrual cycle has been studied in three patients experiencing frequent attacks. Nineteen of their 27 admissions in attack for which...

...G. H. Elder; Diane M. Sheppard; R. E. De Salamanca; A. Olmos 1. Erythrocyte uroporphyrinogen decarboxylase activity has been measured in 27 patients with porphyria cutanea tarda, of whom 11 had a family history of overt porphyria cutanea tarda. 2. Eight patients from six families had erythrocyte...

... 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...

...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...

...B. C. Campbell; M. J. Brodie; G. G. Thompson; P. A. Meredith; M. R. Moore; A. Goldberg 1. The activities of six of the enzymes of haem biosynthesis have been assayed in peripheral blood from patients with lead poisoning, acute intermittent porphyria or hereditary coproporphyria. 2. Compared...

... 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...

...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...

... 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...

...D. C. Nicholson; M. L. Cowger; J. Kalivas; R. P. H. Thompson; C. H. Gray 1. Labelled glycine and/or δ-aminolaevulinic acid (ALA) were administered to a child with congenital erythropoietic porphyria (Günther's disease), to three normal children and to three patients with erythropoietic...

...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...