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Biomedical subjects

H Niessner

Publications and source records attributed to H Niessner.

At least 55 records · Page 3Linked to original sources
PubMed1983

1-Deamino-8-D-arginine-vasopressin--an alternative in the management of mild haemophilia A and von Willebrand's disease.

Intravenous administration of 0.4 micrograms DDAVP/kg body weight in 16 normal controls, 34 patients with haemophilia A and 30 patients with von Willebrand's disease (vWd) was followed by an increase in FVIII: C from 230 to 410%, in FVIIIR:Ag from 160 to 260% and FVIIIR:RC of from 160 to 320%. Additionally, in the patients with vWd, a shortening of the bleeding time and improvement in platelet retention was observed. In 7 haemophiliacs with pretreatment levels of FVIII: C ranging from between 11 and 43% dental extractions were performed successfully after DDAVP whereas in 2 patients with FVIII: C levels of 5 and 6%, respectively, severe bleeding necessitated administration of factor VIII concentrates. In 8 haemophiliacs (FVIII: C between 6.5 and 50%) and 2 patients with vWd (FVIII: C 18 and 36%, respectively) DDAVP enabled minor surgery and successful therapy of spontaneous or traumatic bleeding complications. However, severe postoperative bleeding after stomach surgery in 2 haemophiliacs (FVIII: C 23 and 40%, respectively) and severe menstrual bleeding in one patient with vWd (FVIII: C 15%) required administration of factor VIII concentrates. At present DDAVP therapy should be restricted to minor surgery and non-life-threatening, spontaneous or traumatic bleeding complications in patients with pretreatment FVIII: C levels higher than 10%.

Arginine Vasopressin↗
PubMed1983

T-cell alterations in hemophiliacs treated with commercial clotting factor concentrates.

Various immunological parameters were determined in 46 patients with severe hemophilia A and in 9 patients with severe hemophilia B. All patients were treated over many years with commercial factor VIII or IX concentrates. Patients with severe classic hemophilia had a significantly reduced relative and absolute number of T-helper cells and a significantly increased relative and absolute number of T-suppressor cells. About half of these patients had an inverse T-helper/suppressor cell ratio. Patients with moderate hemophilia A and severe hemophilia B did not show these abnormalities. Hemophiliacs with an inverse ratio had a significantly higher concentration of serum total protein, IgG and IgM. No relationship between the amount of factor VIII concentrate administered, the HLA-type of the patient, the presence or absence of CMV-antibodies, hepatitis markers, thrombocytopenia and abnormal liver function tests to the T-cell abnormalities could be established. Lymphadenopathy was frequently associated with an inverse ratio. Indirect evidence suggests that the alterations of the immune system began in 1979/80.

Acquired Immunodeficiency Syndrome↗
PubMed1982

[Bone marrow transplantation for aplastic anemia--initial results in 8 patients].

8 young patients (aged 11 to 23 years) with severe aplastic anaemia received bone marrow grafts from their HLA-identical, MLC-non reactive siblings. All patients had received repeated transfusions previously and had been unsuccessfully treated with corticosteroids (7 out of the 8 patients) and/or anabolic drugs (4 out of the 8 patients). In order to prevent graft rejection 5 patients received donor buffy coat cells after the marrow infusion and 3 patients underwent total body irradiation with 400 rad prior to the marrow transplantation. 5 patients are alive, 3 patients died. Death occurred from Candida septicaemia (day 4 after transplantation), left ventricular failure (day 14) and graft versus host reaction of the gut (day 85). The 5 living patients are in a very good state of health 30 to 166 days after transplantation. 4 patients already have normal blood cell counts. 2 of the surviving patients developed a transient GVH-reaction of the liver. One patient had a mild GVH-reaction of the skin on day 130.

ABO Blood-Group System↗
PubMed1982

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↗
PubMed1981

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↗
PubMed1980

[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↗
PubMed1979

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↗
PubMed1978

[Splenectomy in idiopathic thrombocytopenic purpura: short- and long-term results (author's transl)].

The results of splenectomy in 25 patients with chronic idiopathic thrombocytopenic purpura (ITP) are reported. Splenectomy was performed when the platelet count was consistently less than 30,000/mm3 in spite of glucocorticoid therapy over an observation period of at least six months. Following splenectomy, 13 patients showed complete remission, 9 partial remission, whilst in 3 cases the condition was unaffected by splenectomy. It is not possible to predict a successful response to splenectomy on the basis of preoperative laboratory findings. A rise in thrombocyte count to over 400,000/mm3 during the first 2 weeks after splenectomy makes complete remission very likely.

Adolescent↗
PubMed1977

[Disorders of blood coagulatiion during haemoperfusion (author's transl)].

Charcoal haemoperfusion of patient may cause a fall in thrombocyte and fibrinogen levels. The possible cause of these changes in haemostasis was investigated by means of five haemoperfusion devices which were filled with 2000 ml ACD fresh blood and perfused for three hours. A continous reduction in the thrombocytes and the plasma coagulation proteins resulted, while the ethanol test and the level of fibrin degradation products stayed within normal levels. These results indicate the adsorption of thrombocytes and protein on to the surface of the charcoal haemoperfusion device. An activation of the coagulation system (i. e. disseminated intravasal coagulation, fibrinolysis) appears unlikely.

Antithrombin III↗
PubMed1977

[Antithrombin III deficiency and tendency to thrombosis (author's transl)].

Antithrombin III (AT III) was determined in 290 patients with deep venous thrombosis and/or pulmonary embolism by immunological methods (radial immunodiffusion, Laurell technique) and by biological activity (heparin cofactor activity and anti-Xa activity). Patients with venous thrombosis had a significantly lower AT III concentration, as determined by the immunological methods or biological method (heparin cofactor activity), than normal persons without any history of venous thrombosis. A decreased level of AT III was found in 27 patients. In these patients the immunoreactive antithrombin III was decreased to the same degree as biological activity (heparin cofactor activity or anti-Xa activity). Thirteen out of these 27 patients belonged to 9 families and, hence, congenital AT III deficiency can be assumed in these cases. The aetiology was unknown in the other half. Patients with AT III deficiency are prone to spontaneous and/or recurrent venous thrombosis. A high incidence of pulmonary embolism and particularly, of fatal pulmonary embolism is remarkable. In more than half of the patients the first thrombotic event occurred before the age of 35. The treatment of choice in such patients is with oral anticoagulants of the coumarin group.

Adult↗
PubMed1977

Coagulation abnormalities in liver disease.

The decreased capacity of the liver to synthesize proteins is the main cause of decreased blood levels of clotting factors II, V, VII, IX, X and of antithrombin III in patients with liver disease. Therefore, determination of the activity or concentration of these coagulation proteins is a useful test of liver function and guide to prognosis, provided that other mechanisms which may influence the blood level are carefully considered. Clotting factor assays have an only limited value for the differential diagnosis in liver disease.

Acute Disease↗
PubMed1977

[The disseminated intravascular coagulation. Diagnosis and therapy on a medical intensive care unit (author's transl)].

Disseminated intravascular coagulation (DIC) is a frequent acquired disorder of haemostasis in the patients at the medical intensive care unit. The pathogenesis, the different possible clinical manifestations and the obligatory laboratory tests are reviewed. In addition to the treatment of the underlying disease, the importance of adequate restoration of fluid volume and early administration of heparin is stressed.

Adolescent↗
PubMed1976

[Synovectomy in the prevention of recurrent joint bleedings in haemophilia (author's transl)].

Synovectomy was performed in seven patients with severe haemophilia A, one with moderately severe haemophilia A and one with severe haemophilia B. Frequently recurring haemorrhages into one knee and demonstrated hypertrophic synovitis were the indications for operation in all. No serious bleeding complications occurred during or after the operations. After synovectomy the frequency of haemarthroses in the synovectomised joint decreased significantly. Satisfactory joint function was eventually achieved in all by gymnastic exercises and physiotherapy over a long period. The operation should only be done in large haemophilia centres were there is sufficient experience.

Adolescent↗