Results 161 to 170 of about 1,644 (213)
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Human hereditary hepatic porphyrias
Clinica Chimica Acta, 2002The human hereditary hepatic porphyrias are diseases due to marked deficiencies of enzymes in the heme biosynthetic pathway. Porphyrias can be classified as either hepatic or erythroid, depending on the major production site of porphyrins or their precursors.
Yves, Nordmann, Hervé, Puy
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Safety of anticonvulsants in hepatic porphyrias
Neurology, 1981Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione.
N C, Reynolds, R M, Miska
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Hepatic porphyrias: diagnosis and management
Clinics in Liver Disease, 2004Porphyrias are a group of metabolic disorders in which there are defects in the normal pathway for the biosynthesis of heme, the critical prosthetic group for numerous hemoproteins. The clinical manifestations of the porphyrias can be highly varied, and patients may present to general physicians and be referred to a wide variety of subspecialists ...
Annie T, Chemmanur, Herbert L, Bonkovsky
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Hepatic porphyria: A narrative review
Indian Journal of Gastroenterology, 2016Porphyrias are a group of metabolic disorders, which result from a specific abnormality in one of the eight enzymes of the heme biosynthetic pathway. These have been subdivided based on the predominant site of enzyme defect into hepatic and erythropoietic types and based on clinical presentation into acute neurovisceral and cutaneous blistering ...
Sumant, Arora +3 more
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La Revue de medecine interne, 1999
This review is aimed at presenting classification and diagnosis criteria of hepatic porphyrias and at proposing guidelines for diagnosis and management of these diseases.Porphyrias are inherited disorders: each type of porphyria is the result of a specific decrease in the activity of one of the enzymes of heme biosynthesis.
Y, Nordmann, H, Puy, J C, Deybach
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This review is aimed at presenting classification and diagnosis criteria of hepatic porphyrias and at proposing guidelines for diagnosis and management of these diseases.Porphyrias are inherited disorders: each type of porphyria is the result of a specific decrease in the activity of one of the enzymes of heme biosynthesis.
Y, Nordmann, H, Puy, J C, Deybach
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Resin Hemoperfusion in Hepatic Porphyrias
Artificial Organs, 1985Abstract: In three types of hepatic porphyrias, 32 hemoperfusions over polyhema‐coated Amberlite XAD‐2 resin were performed. An attack of acute intermittent porphyria subsided after a 6‐h hemoperfusion. During the procedure, 13 L of plasma was completely cleared of porphyrins. In a child with variegate porphyria, the laboratory changes were slight and
J, Horák +6 more
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Hereditary Hepatic Porphyrias in Finland
Acta Medica Scandinavica, 1976ABSTRACT. The occurrence of hepatic porphyrias —acute intermittent porphyria (AIP) and variegate porphyria (VP)—in Finland has been studied. During a period of 9 years 107 patients with AIP and 45 patients with VP were found. The prevalence of hereditary hepatic porphyrias was calculated to be 3.4 per 100 000 inhabitants.
P, Mustajoki, P, Koskelo
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The “glucose effect” in acute hepatic porphyrias and in experimental porphyria
Klinische Wochenschrift, 1981The "glucose effect" was investigated in human acute hepatic porphyrias (acute intermittent porphyria, variegate porphyria, coproporphyria and porphobilinogen synthase defect porphyria) and in avian liver cells. 8 patients (7 women) with acute abdominal-neurological porphyria syndrome and 3 patients (2 women) in the remission phase were treated with ...
M, Doss, F, Verspohl
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Chronic hepatic porphyria in chronic aggressive hepatitis
Klinische Wochenschrift, 1971An acquired (“symptomatic”) chronic hepatic porphyria (CHP) is described, which develops secondarily following damage to the liver tissue and is characterized by the absence of clinical symptoms. It can be differentiated biochemically from latent porphyria cutanea tarda (PCT) by a lower total porphyrin excretion (0.18–0.64 mg/l) and a relatively ...
M, Doss, D, Look, H, Henning
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Minerva medica, 1979
Porphyria is making increasing demands on the attention of clinicians and research worker. An account is given of hepatic forms, since these have recently come into prominence on account of recent advances in the understanding of their metabolic, diagnostic and therapeutic aspects.
G, Tornaghi, L, Vitali
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Porphyria is making increasing demands on the attention of clinicians and research worker. An account is given of hepatic forms, since these have recently come into prominence on account of recent advances in the understanding of their metabolic, diagnostic and therapeutic aspects.
G, Tornaghi, L, Vitali
openaire +1 more source