Porphyric polyneuropathy and its pathogenesis in the light of electrophysiological investigations.

Twenty-four patients with acute intermittent porphyria were examined by means of routine electromyographic (EMG) tests, measurements of motor and sensory conduction in peripheral nerves and stimulation fatigue tests. The EMG results gave evidence of an initial, reversible disorder of function of the peripheral neuron, only sometimes followed by evidence of axonal degeneration of the "dying back" type. In stimulation fatigue tests there was some evidence of potentiation but none of fatigue. Attention is drawn to some analogies between the electrophysiological changes in porphyric polyneuropathy and in botulism. It seems that the pathogenesis of porphyric polyneuropathy may be dependent upon a block of cholinergic endings.

Blood substitutes.

With the development of modern methods of surgery, anaesthesia, and pre- and postoperative care the requirement for blood substitutes is continuously increasing. We present a review of the different blood substitutes which are already in clinical use or in an advanced stage of experimental investigation for possible practical administration. Our own clinical experience with dextrans and experimental studies on stroma-free haemoglobin and hydroxyethyl starch solutions are described.

[Neurologic disorders in acute intermittent porphyria]. / Zaburzenia neurologiczne w ostrej przerywanej porfirii.

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.

[Social problems of porphyria]. / Problemy spoleczno--socjalne w porfirii.

Late consequences of the attacks of acute porphyrias were studied. Among 312 patients who were investigated the motor disability (neuropathy) was found in 9 patients, psychoneurological changes in 1 case and drug abuse in 7 subjects. In the group of 200 patients in remission (chosen at random) 72 persons (36%) received a disability pension. The existence of problems in change of the profession when it was necessary for health-reasons was marked. It was find that Dolargan (Pethidine) is too often applied to the patients in remission. It was made the warning that drug dependence will arise very easy in patients with porphyria.

[[35 Years of effort to improve the diagnosis of porphyria]. / 35 lat staran o lepsze rozpoznawanie porfirii.

World history of porphyria is given in brief. The activities of Porphyria Center during period of 25 years have shown how difficult it was to introduce diagnostic measures for this rare and very little known group of diseases. The obstacles encountered during the popularization of the knowledge of porphyria were: minimal information on porphyria, very seldom seen in Poland, therefore rather negative approach of the doctors to these patients,--almost never porphyria was seen in differential diagnostics of abdominal pains or polyneuropathies, herefore diagnosis, if made, was very late, often just before death. From the other side insufficient support for doctors was given by laboratories because of the lack of simple diagnostic tests. Along the 35 years of the work in the Institute of Haematology and Transfusiology the situation was steadily improving but slowly due to technical and economical difficulties. The Center was organized, now well equipped and able to diagnose and differentiate all types of porphyrias. A computerised basis of the collected material consist of 383 families (6000 persons registered). Among them there are nearly 600 acute (who have passed one or more attacks of porphyria) and about 1000 latent cases of porphyria. The mortality which was 52% in the first collected group of patients (1960-1970) has fallen to less than 10% concerning very severe and late diagnosed attacks. In 1996-1998 there were only two deaths during attacks of porphyria in Poland.

[The course of acute hepatic porphyria]. / Przebieg ostrych porfirii watrobowych.

Acute hepatic porphyrias are the diseases dependent on the congenital metabolic defects of the hem biosynthesis. The diagnosis of porphyria only on the grounds of clinical picture is extremely difficult as it is presented by the variety of symptoms, often imitating other diseases. In each case the diagnosis must be confirmed by the biochemical and enzymatic investigations. In the paper we described different clinical courses of the acute hepatic porphyrias. The acquaintance with the symptoms of porphyrias enables to decrease the number of complications connected with diagnostic and therapeutic errors what is still a serious economic problem.

[Anti - T. gondii antibody concentration in sera of pregnant women in the sample of Lódz population]. / Stezenie przeciwcial anty -T. gondii w surowicy kobiet ciezarnych w próbie populacji regionu lódzkiego w roku 1998.

The aims of the study were: to estimate the prevalence of Toxoplasma gondii in pregnant women in the Lódz region; to assess the rate of incidence of suspected recent infection based on the results of the standard test for specific anty -T. gondii IgG and IgM antibodies. The study covered 1920 pregnant women served in 1998 by Toxoplasma Reference Laboratory in Lódz. T. gondii specific IgG and IgM were quantitated by an enzym-linked immunoabsorbent assay (ELISA, Organon). We have demonstrated high prevalence of T. gondii among pregnant women in the Lódz region. In the analysed sample of Polish population the T. gondii-specific IgG with negative IgM occurred in 43.4% (n = 837). In 1.42% of pregnant women IgG and IgM specific antibodies were detected.

[Effect of gelatin and dextran infusion on the reticuloendothelial system]. / Wplyw przetaczania preparatu zelatyny i dekstranu na uklad siateczkowo-sródblonkowy.

The purpose of this study was evaluation of the phagocytic ability of peripheral blood leucocytes and reticuloendothelial system of the lungs, liver and spleen after one infusion of plasma-substituting preparations: Fluigel and dextran 70 in volumes of 20 ml/kg of body weight. The experiments were done in rabbits. The response of the reticuloendothelial system was assessed on the basis of its ability of eliminating foreign erythrocytes labelled with 51Cr from the circulating blood and radioactivity accumulation in the liver and spleen. It was found that infusion of Fluigel and dextran produced transient changes in the function of the reticuloendothelial system with increased number of granulocytes and a fall of their radioactivity 3 and 6 hours after infusion, reduced ability of pulmonary 51Cr accumulation and increased storage in the spleen observed 24 hours after infusion.

[Factors inducing clinical symptoms of acute hepatic porphyria 1986-1990]. / Przyczyny indukujace objawy kliniczne ostrych porfirii watrobowych w latach 1986-1990.

The most frequent cause of porphyria attacks in this time period were drugs used in symptomatic treatment (in 69 out of 195 cases). In 42 women attacks were induced by sex hormone disturbances, in 21 cases by contact with paints and lacquer in 7 by alcohol and in 3 by calorie deficiencies. The main cause of porphyria attacks in carriers of this metabolic error is lack of porphyria considering in the differential diagnosis of abdominal pains, polyneuropathy and vague mental disorders, which leads to erroneous treatment worsening the course and prognosis of the disease.

[Effect of stroma-free hemoglobin solution on oxygen consumption in some tissues]. / Wplyw roztworu hemoglobiny pozbawionej zrebu komórkowego na zuzycie tlenu w niketórych tkankach

50 cc of a 6%, oxygenated or non oxygenated stroma-free heamoglobin solution was transfused in rabbits after previous removal of about one third of the circulating blood volume. The experimental investigations were carried out on animals under barbiturate anaesthesia. Oxygen consumption was determined using a Warburg microrespirator. Posthaemorrhagic hypotension developing after bleeding caused a significant increase in oxygen consumption by liver tissue. Transfusion of non oxygenated stroma-free haemoglobin solution caused a further increase of oxygen consumption, while transfusion of oxygenated stroma-free haemoglobin solution slightly decreased oxygen consumption compared to the values observed during the post haemorrhagic hypotension.