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Biomedical subjects

K Lechner

Publications and source records attributed to K Lechner.

At least 379 records · Page 21Linked to original sources

Systemic candidiasis complicating bone marrow transplantation in aplastic anemia. Case report.

A 17-year-old male patient with aplastic anemia underwent bone marrow transplantation and succumbed 4 days after marrow infusion from sudden myocardial failure. Fever of unknown origin (FUO) had accompanied the patients course from admission until death. The cause of death was fungus myocarditis, which had escaped detection in vivo, in spite of a daily culture program for bacteria and fungi, and a close monitoring of the patients circulation and ventricular performance. Commonly applied diagnostic criteria for systemic mycosis, such as topical colonization, malfunction of invaded organs and positive fungus cultures failed to provide a timely diagnosis. With regard to the problems in diagnosing systemic mycosis, the potential stem cell toxicity of antifungal drugs and the need for immunosuppressive therapy prior to marrow infusion, we strongly recommend not to start the transplantation procedure unless FUO has been treated successfully.

Adolescent↗

Plasmapheresis: its value in the management of patients with antibodies to factor VIII.

12 plasmapheresis were carried out in 5 patients with antibodies to F VIII (3 haemaophilic antibodies; 2 spontaneous antibodies). Plasmapheresis led in all instances to a marked reduction of the antibody level there was a good correlation between the amount of plasma exchanged and the decrease of the antibody level. About 40 ml of plasma/kg body weight have to be removed to reduce the antibody level to half. In patients with low titre antibody who need treatment for serious bleeding, plasmapheresis is a more rapid and less expensive procedure than neutralisation of the inhibitor by high doses of F VIII. In one haemophiliac repeated plasmapheresis and subsequent high dose F VIII treatment eliminated the antibody within a short time. Plasmapheresis should always be considered when patients with antibodies to F VIII have to be treated because of severe bleeding.

Adolescent↗

Glycosylated hemoglobins (GHb): an index of red cell survival.

Levels of glycosylated hemoglobins (GHb) are significantly (p less than 0.0005) lower in patients with hemolytic anemia (n = 20; mean = 3.9% +/- 0.1% SD GHb of total Hb) compared to patients with nonhemolytic anemia (n = 20; mean = 7.0% +/- 0.7% GHb) and normal controls (n = 30; mean = 6.7% +/- 0.7% GHb). A curvilinear correlation between GHb and red cell survival is demonstrable (n = 20;r2 = 0.88; p less than 0.001). Determination of GHb may be useful as a screening test for hemolytic anemia and for the evaluation of the degree of hemolysis, provided that diabetes mellitus can be excluded.

Adolescent↗

A survey of 215 non-hemophilic patients with inhibitors to Factor VIII.

Information was obtained by questionnaire about 215 non-hemophilic patients who developed inhibitors against factor VIII (antihemophilic factor). The majority of the patients were over 50 years of age, and approximately equal numbers of males and females were reported. Rheumatoid arthritis was present in 8% of the cases, 7% occurred during pregnancy or the post-partum period, and in several there was an association with allergy to penicillin, asthma, "auto-immune" diseases, or malignancy. In 46% of cases, no underlying disorders were identified. Major bleeding was observed in 87% of patients, and in 22%, death was attributed either directly or indirectly to the presence of the inhibitor. In 11 of 31 patients receiving no therapy other than supportive transfusions of blood or factor VIII concentrate, the inhibitor disappeared after being present for an average duration of 14 months. Corticosteroids were thought to be effective in abolishing the inhibitor in 22 of 45 patients in whom these were the only drugs administered. Twenty-eight patients received azathioprine as well as corticosteroids; in 19, the inhibitor declined or disappeared during treatment. Finally, 80 patients were treated with cyclophosphamide; in 37 there was a favorable outcome. Inhibitors in children and post-partum patients were more likely to disappear spontaneously or with steroid therapy, whereas those in patients with rheumatoid arthritis or other "autoimmune" disorders required treatment with alkylating agents. However, before any specific therapy can be recommended for this disorder, prospective trials of potential therapeutic agents should be conducted in selected subgroups.

Adolescent↗

Peripheral nerve lesions in haemophilia.

Among 234 haemophiliacs (A and B) treated between 1948 and 1975 at the First Medical Clinic of the University of Vienna, 25 (10.8%) with 36 peripheral nerve lesions have been observed. This amounts to 15.3% or 2.4 lesions within 100 observation years. The average age of the patients at the time of development of lesion was 26.4 years. Correlations between severity of haemophilia and frequency of occurrence of lesions were found. Patients with mild haemophilia did not show peripheral nerve disturbances. The numbers of times individual nerves were affected are as follows: maxillary (1), radial (2), medial (6), ulnar (4), obturator (1), lateral cutaneous nerve of thigh (1), femoral (12), including lesions of the 2nd and 3rd lumbar roots, sciatic (6), including lesions of te 5th lumbar and the 1st sacral roots, peroneal (3), and tibial (1). In 1978, 11 patients who previously had sustained 16 peripheral lesions were reexamined. Of these, 10 had fully recovered, 4 showed some persisting sensory disturbances, and 2 were unchanged. Motor nerve conduction velocity, distal latency and compound nerve action potentials were systematically examined in 11 cases on both sides of the body. In contrast to our expectations, no signs of subclinical peripheral nerve lesions were found. The comparability of the presence and previous results is discussed.

Adolescent↗

Plasma concentration of platelet-specific proteins and fibrinopeptide A in patients with artificial heart valves.

beta-Thromboglobulin (beta TG), platelet factor 4 (PF4), fibrinopeptide A (FPA), lactic dehydrogenase (LDH), and platelet count were evaluated in patients with bioprostheses and prosthetic heart valves. beta TG and PF4 were significantly elevated in both patient groups (p less than 0.001), whereas FPA was normal. There was no significantly difference in plasma concentrations of beta TG and PF4 between patients with prosthetic heart valves and bioprostheses. LDH levels were significantly (p less than 0.001) higher and platelet count lower (p less than 0.001) in patients with prosthetic cardiac valves. The data indicate that bioprostheses do not cause haemolysis or activation of the coagulation system. The findings that the plasma concentration of platelet-specific proteins were elevated, support the assumption that both types of valves cause platelet damage.

Adult↗

Post-transfusion thrombocytopenic purpura: immunological and clinical studies in two cases and review of the literature.

Two patients with post-transfusion thrombocytopenic purpura are described, the first cases recognized in Austria and Germany. Both patients were female, 51 and 60 years of age. The purpura was evoked by blood transfusions with a latent period of 2 and 7 days. The thrombocytopenic episode lasted for 22 and 60 days. In one case, thrombocytopenia was associated with agranulocytosis due to transient bone marrow failure. Both patients were negative for the platelet-specific antigen PlA1 (= ZWa). Their sera contained PlA1 as well as potent HLA antibodies. Detailed clinical and serological data are presented. The literature with a total of 25 cases so far reported is surveyed.

Agranulocytosis↗

[Amaurosis fungax. Studies on hemodynamics and platelet function].

Thirty-eight patients with amaurosis fugax were examined ophthalmologically. In 12 of these cases carotid occlusion was diagnosed by ophthalmodynamography and Doppler ultrasonography of the orbital vessels. Angiographic substantiation was possible in nine of these patients. The pathognomonic importance of amaurosis fugax for occlusive carotid disease is clearly shown by these findings. The plasma concentration of platelet specific proteins was examined in 32 patients and found to be pathologically increased in 13 cases. Spontaneous aggregation of platelets and circulating platelet aggregates were only found in a few patients. Due to the lack of coincidence between the event of amaurosis fugax and the time when platelet function tests were made no definite conclusions may be drawn from these results. Yet disturbances of platelet function must be taken into consideration in the pathogenesis of amaurosis fugax.

Adult↗

The German-Austrian aspirin trial: a comparison of acetylsalicylic acid, placebo and phenprocoumon in secondary prevention of myocardial infarction. On behalf of the German-Austrian Study Group.

In a multicenter clinical trial on the prevention of recurrent myocardial infarction, 946 patients who had survived a myocardial infarction for 30-42 days were randomly allocated to acetylsalicylic acid (ASA, 1.5 g/day) (317 patients), placebo (309 patients) or phenprocoumon treatment (320 patients) and were followed to determine the incidence of total mortality, coronary death and nonfatal recurrent myocardial infarction. The ASA and placebo groups were treated in double-blind fashion. The observation period for each patient was 2 years. Total mortality was lower in the ASA group (27 patients) than in the placebo (32 patients) and phenprocoumon groups (39 patients). There were 13 coronary deaths (fatal myocardial infarction and sudden death) in the ASA group, 22 in the placebo group and 26 in the phenprocoumon group. This represents a reduction rate of 42.3% in the ASA group compared with placebo (p less than 0.1) and of 46.3% in the ASA group with phenprocoumon (p approximately 0.07). Considering male patients alone, the difference regarding coronary death is significant between ASA vs placebo (p less than 0.05, reduction rate 56.4%) and ASA vs phenprocoumon (p less than 0.05, reduction rate 55.6%). Coronary events (coronary death and nonfatal recurrent myocardial infarctions) were lower in the ASA group (24 events) than in the placebo (37 events) (p less than 0.07) or phenprocoumon group (32 events).

4-Hydroxycoumarins↗

[Rational substitution therapy in blood coagulation disorders].

Indications and possible side effects of clotting factor concentrates are reviewed. Although clotting factor concentrates are natural products, their side effects can not be neglected. When using clotting factor concentrates the risk/benefit ratio has to be always considered.

Anemia, Hemolytic, Autoimmune↗

Effect of treatment with activated prothrombin complex concentrate (FEIBA) on factor VIII-antibody level.

The influence of treatment with an activated prothrombin complex preparation (FEIBA) on the antibody level was studied in 10 haemophiliacs with an antibody to factor VIII. The antibody level was observed to rise at least once in five patients, while in the remaining five patients no rise occurred. In all, 6 out of 31 treatments were followed by an anamnestic rise of the antibody level, corresponding to 19.4%. A rise of the inhibitor level following FEIBA treatment is likely to occur in patients who show a marked antibody rise after factor VIII treatment (good responders), but have a low antibody level at the time of treatment. High doses of FEIBA and simultaneous of red cells may also enhance the likelihood of an anamnestic response. Stimulation of antibody production is probably due to the presence of small amounts of factor VIII in this preparation.

Antibodies↗

HLA antigens in immunologic thrombocytopenic purpura (ITP).

79 patients with definite ITP were investigated for HLA-A, -B, -C antigens. There was an increased frequencey of HLA-B5 in the entire groups (27.8% vs. 16.5% in controls and for HLA-B12 in male patients. However, after correction for the number of antigens tested, the difference of antigen frequencies became insignificant.

Adolescent↗