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Biomedical subjects

E Kostrzewska

Publications and source records attributed to E Kostrzewska.

At least 19 recordsLinked to original sources
PubMed2001

[Uniform programme in caring for patients with acute hepatic porphyria].

The programme commissioned by the Ministry of Health is presented. Data basis for the years 1996-1998 were created. A booklet on diagnosis, treatment and prophylaxis of porphyrias was edited and distributed in Poland in 5000 copies to all hospitals, major laboratories, medical libraries, regional officers as well as patients. The booklet lists simple diagnostic methods which can be easily introduced to hospital laboratories for early diagnosis of porphyria. It also includes a list of the safe drugs for patients with porphyria. Several lectures for doctors and medical staff were delivered, nine publications appeared and six others were accepted for publication. Results of the work, evaluated after three years are as follows. Early clinical diagnosis of porphyria and its prophylaxis among family members has significantly improved. The frequency of severe attacks and the mortality rate due to porphyria has been reduced to singular cases. The proposed organization of small regional centers (with help of Porphyria Center), oriented at early diagnosis of porphyria appears to be impossible without further financial support.

Guidelines as Topic↗
PubMed2001

[Anti - T. gondii antibody concentration in sera of pregnant women in the sample of Lódź population].

The aims of the study were: to estimate the prevalence of Toxoplasma gondii in pregnant women in the Lódź 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 Lódź. 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 Lódź 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.

Adolescent↗
PubMed1999

[[35 Years of effort to improve the diagnosis of porphyria].

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.

Hematology↗
PubMed1998

[The course of acute hepatic porphyria].

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.

Acute Disease↗
PubMed1998

[Social problems of porphyria].

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.

Adult↗
PubMed1994

Coexistence of hereditary coproporphyria with acute intermittent porphyria.

A new form of acute hepatic porphyria with double genetic defect--deficiency of porphobilinogen deaminase and coproporphyrinogen oxidase--is described. Among 17 studied family members this double enzymatic deficiency was found in five individuals, deficiency of porphobilinogen deaminase in four, and deficiency of coproporphyrinogen oxidase in two. Only the proband had an attack of porphyria. Apart from the proband, all family members had normal urinary PBG excretion. Increased faecal coproporphyrin excretion was found in three people. The results obtained suggest that deficiency of porphobilinogen deaminase and coproporphyrinogen oxidase can be inherited independently. coproporphyrinogen oxidase can be inherited independently.

Adolescent↗
PubMed1991

[Acute intermittent porphyria and arterial hypertension].

A group of 40 female patients with acute intermittent porphyria from 5 to 34 years after attacks of porphyria were examined. In two patients arterial hypertension developed before attack. In 18 cases hypertension was observed in different periods of time after attack. The comparison of these findings with epidemiological data of similar group of the Polish population suggests that arterial hypertension develops earlier and more frequently in female patients with acute intermittent porphyria. Periodic control of blood pressure in patients with acute intermittent porphyria is proposed.

Acute Disease↗
PubMed1991

[Factors inducing clinical symptoms of acute hepatic porphyria 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.

Acute Disease↗
PubMed1986

Increased activity of porphobilinogen deaminase in erythrocytes during attacks of acute intermittent porphyria.

The activity of porphobilinogen deaminase was determined in 25 patients with acute intermittent porphyria during and after fully developed attacks of porphyria. It was found that in most cases (in 20 of 25) it was higher than 24.3 nmoles/ml erythrocytes/hour, a value considered as characteristic for acute intermittent porphyria, and that it decreased during convalescence and remission. In a proportion of these cases the decrease in the activity of the enzyme was parallelled by decreasing urinary excretion of porphobilinogen. A normal activity of porphobilinogen deaminase during an attack of porphyria can be a source of error in the differential diagnosis of porphyria.

Acute Disease↗
PubMed1984

The function and morphology of the liver in porphyria cutanea tarda.

The aminopyrine breath test, postprandial serum bile acids, and routine liver tests were assessed as indicators of liver dysfunction in 38 patients with porphyria cutanea tarda. In 17 patients needle biopsy specimens of the liver were obtained. Bile acids were increased in almost all the patients studied (97%) but impairment of aminopyrine demethylation was found in only 45%. More than 50% of cases had elevated activities of serum alanine aminopeptidase, leucine aminopeptidase, gammaglutamyl transpeptidase (GGTP), and alanine aminotransferase (ALAT). All liver biopsy specimens showed fluorescence characteristic of porphyrins. Histologic examination of biopsy material revealed cellular lesions in all cases, the most common pathological findings being fatty degeneration of varying degree and iron accumulation. The most frequent electronmicroscopic changes in the liver were fat droplets, granules containing bile material, and siderosomes in the cytoplasm of hepatocytes. However, there was no evident relationship between morphological and functional liver changes.

Adult↗