Biomedical subjects

A Gregor

Publications and source records attributed to A Gregor.

183 records · Page 11Linked to original sources

PubMedDate not supplied

[Neurologic disorders in acute intermittent porphyria].

In a material of 28 patients with acute intermittent porphyria and 2 with mixed porphyria neurological disturbances occurring in acute attach and the degree of their persistence during remission were analysed. The obtained results indicate that the symptoms of acute attach are neurogenic. In the acute phase of the disease a reversible disturbance of function develops in the first place in the nerve fibres with acetylcholine-type of impulse transmission. Clinically detectable permanent signs of polyneuropathy were observed only in some cases with most severe and prolonged acute attack.

Acute Disease↗

PubMedDate not supplied

[Porphyrin fluorescence in plasma of various types of porphyria].

Fluorescence spectra of plasma porphyrin were measured in 6 patients with the acute intermittent porphyria (AIP), 30 patients with variegate porphyria (PV), 2 patients with hereditary coproporphyria, 2 patients with porphyria cutanea tarda, and in 6 patients with erythropoietic protoporphyria (EPP). It was found that the excitation and emission wavelengths at which maximum fluorescence is seen may help to diagnose and differentiate PV and EPP. In patients with AIP spectrum characteristic for porphyria of such a type was noted in all patients during the attack of disease and in only 33% of patients in remission. Fluorescence spectrum was normal in asymptomatic family members. In variegate porphyria spectrum with a characteristic maximum of fluorescence was noted in all patients during an attack and remission, and 62% of the asymptomatic family members.

Diagnosis, Differential↗

PubMedDate not supplied

Acute hepatic porphyrias. Detection, prophylaxis and treatment.

Between 1962 and 1944, 300 families with acute hepatic porphyrias were seen at the Porphyria Centre in Warsaw. Among members of these families, 443 overt and 707 latent cases were diagnosed, and 211 persons had intermediate values of enzyme activity without clinical symptoms and biochemical changes in urine and stool. Three dual porphyrias were detected. Five children with acute symptoms were also seen. The patients and their relatives were registered and routine prophylactic measures were introduced. The treatment of the attacks was based on glucose and hemearginate infusions, a high carbohydrate diet and maintenance of body homeostasis. As a late consequence of the attack acute intermittent porphyria arterial hypertension was observed.

Acute Disease↗