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Biomedical subjects

H Stormorken

Publications and source records attributed to H Stormorken.

At least 73 records · Page 4Linked to original sources

Hemophilia BM in a female.

A 28 year old pregnant woman was referred for genetic counselling because of a bleeding tendency and a family history of hemophilia. The hemophilia patients had 0.02 units/ml of factor IX activity and a normal concentration of factor IX antigen. In addition they had a prolonged coagulation time with bovine thromboplastin and were therefore cases of hemophilia BM. At the age of six the patient was hospitalized because of prolonged bleeding after a tooth extraction. At the age of 20 and 24 she gave birth to healthy daughters. The first delivery was complicated by a serious bleeding seven days post partum whereas the second delivery was without complications. Factor IX activity when she was three months pregnant was 0.02-0.03 units/ml and the factor IX antigen concentration was normal. Coagulation time with bovine thromboplastin was prolonged. Delivery was again normal, and she had a daughter with carrier values of factor IX. Her mother also had carrier values whereas her father was normal. The patient's hemophilia BM was probably due to extreme Lyonization in a heterozygote.

Adult↗

A new syndrome: thrombocytopathia, muscle fatigue, asplenia, miosis, migraine, dyslexia and ichthyosis.

A new multifacetted syndrome inherited as an autosomal, dominant trait is described encompassing not only two hitherto undescribed hereditary defects--thrombocytopathia and asplenia--but also muscle contractile defect, migraine-like headache, miosis, dyslexia and ichthyosis. None of these defects has so far been assigned to a specific chromosome or linkage group. Further studies on the various aspects of the syndrome are in progress.

Child↗

Haemostatic studies in osteogenesis imperfecta.

Two-thirds of osteogenesis imperfecta (OI) patients claimed that they bruised easily. We have studied haemostasis in 58 subjects with OI, tarda type. The most frequent abnormalities were increased capillary fragility (35%), decreased platelet retention (33%) and reduced factor VIII R:Ag (23%). Reduced ristocetin cofactor, deficient platelet aggregation induced by collagen and prolonged bleeding time were less common findings. The combination of vascular, platelet related and plasmatic defects may reflect that OI is a heterogeneous group of disorders with common clinical expression. The tourniquet test is the most valuable screening test of the haemostatic defects observed in OI.

Adolescent↗

Species specificity of tissue thromboplastin.

The species specificity of thromboplastin (tissue factor) is an important fact to take into consideration when clotting assays or experiments are planned. We have (re)investigated all possible combinations of thromboplastin and plasma from several of the most commonly used species of experimental animals.

Animals↗

The value of activated coagulation time in monitoring heparin therapy during extracorporeal circulation.

The anticoagulant effect of heparin was studied in 20 patients undergoing aortocoronary bypass surgery. The protamine dose necessary to reverse heparin after extracorporeal circulation (ECC) was assessed in ten patients from individual heparin dose-response curves (HDR group). The other ten patients received protamine according to a routine protocol (control group). The protamine administration was followed in both groups by injection of 3-5 g tranexamic acid (Cyklokapron). A wide range of sensitivity to heparin was shown by the patients. Although almost twice as much protamine was given to the control group as to the HDR group, the effect on heparin reversal was similar. The variability of protamine dose did not influence the post-bypass levels of fibrinogen, AT-III, activated coagulation time (ACT) or Cephotest, and fibrinolysis was not observed in either of the groups. During ECC there was poor correlation between ACT and plasma heparin levels, and the use of heparin dose-response curves was grossly misleading in regard to true heparin concentration. The postoperative bleeding was not related to the levels of heparin or coagulation parameters after heparin reversal. The concentrations of fibrinogen and AT-III showed variations dependent on the changes in haematocrit. A number of factors other than heparin that influence ACT are discussed.

Aged↗

A new dysfibrinogenemia: fibrinogen Oslo IV.

A family with dysfibrinogenemia is described. The abnormal fibrinogen occurred in three successive generations indicating a dominant hereditary pattern. Thrombin and reptilase times were about twice the normal value. This was shown to be caused by a polymerization defect, fibrinopeptide release being normal. Platelet aggregation was undisturbed, indicating normal platelet-fibrinogen binding. The bleeding time was normal and there was no bleeding tendency. However, an obscure recurrent pulmonary ailment may, or may not, be related to the dysfibrinogenemia. The abnormal fibrinogen was tentatively termed Oslo IV.

Amino Acid Sequence↗

A new bleeding disorder: lack of platelet aggregatory response to adrenaline and lack of secondary aggregation to ADP and platelet activating factor (PAF).

A bleeding disorder, probably familial, with absent adrenaline-induced platelet aggregation and lack of secondary aggregation response to ADP and platelet activating factor (PAF), is described. The laboratory findings do not fit any hitherto recognized hemorrhagic disease. The disorder was not caused by alpha-adrenergic receptor deficiency, but the ultimate defect has not yet been unraveled. This patient illustrates that a normal response to more than one aggregating stimulus is necessary for normal hemostasis, and indicates a physiopathological role for adrenaline not hitherto recognized. Whether this also applies to PAF remains to be proven.

Adenosine Diphosphate↗

Tolerability of iohexol after injection into healthy volunteers.

The effects of iohexol, a new non-ionic contrast medium, after intravenous injection into humans are reported. After injection of small doses into 2 subjects, iohexol was injected intravenously into 20 healthy male volunteers in doses of 125 to 500 mg I/kg body weight. A large number of physiologic, biochemical, hematologic and pharmacokinetic parameters were analysed. The results indicated that iohexol was well tolerated and that clinical trials in patients could be undertaken.

Adult↗

Diagnosis of heterozygotes in Glanzmann's thrombasthenia.

A study of a family with a propositus suffering from classical thrombasthenia type I has shown that the new immunochemical methods detect heterozygotes with high reliability. There was no overlapping between heterozygotes and normals, and the concentration of the glycoproteins IIb-IIIa-complex is remarkable constant around 50-60% in the heterozygotes. Furthermore, heterozygotes as a group show an increased bleeding tendency.

Adult↗

Platelet function related to the development and presenting symptoms in coronary artery disease. An epidemiological study in apparently healthy men.

Among 2014 apparently healthy men aged 40-59 years platelet function was studied with Hellem's retention method in 488 men. In 55 of these 488 men coronary heart disease (CHD) was strongly suspected and coronary angiography confirmed the diagnosis in 34 of 51 angiographied men. Platelet responsiveness was significantly lower among those who had angina pectoris than among their CHD-suspect counterparts without angina, regardless of coronary angiographic findings. Since angiopositive men without angina and 408 "normals" had similar retention values, it seems as if presenting symptoms, but not the development of CHD are linked to platelet function. During a near maximal bicycle exercise test significantly higher peak exercise blood pressure was found among men with very low retention values than among men with retention values in the middle and upper range. This could not be explained by differences in maximal heart rate or work performance. This indicates that blood pressure regulation during exercise may in part be linked to platelet function related mechanisms.

Adult↗

Acute iron intoxication with abruptly reduced levels of vitamin K-dependent coagulation factors.

A 17-year-old girl swallowed at least 50 Duroferon duretter. Each tablet contains ferrous sulphate equivalent to 0.1 g Fe2+. Thus, a total of 5 g Fe2+ was ingested. Vitamin K-dependent coagulation factors dropped within the next hours to very low levels. Thrombotest showed less than 3% of normal coagulation activity 8 h after oral intake. Recovery was uneventful except for laboratory evidence of transient liver damage. The rapidity by which the early coagulation deficiencies developed and the lack of evidence of disseminated intravascular coagulation suggested a direct effect of iron on coagulation factors. In vitro studies confirmed that iron in concentrations that may have been attained in vivo, altered the functional activity of several coagulation factors. Monitoring the vitamin K-dependent coagulation factors may be a simple and useful parameter in acute iron intoxication.

Acetaminophen↗

ABO blood groups and coronary heart disease (CHD). A study in subjects with severe and latent CHD.

The view based on epidemiological and laboratory data that blood group A subjects (=A) have clinically significant higher thrombotic potential than blood group 0 subjects (=O), is supported by the present finding of a significantly higher platelet retention in A than 0. The completely normal AB0 distribution found among 71 cases of proven latent CHD, and the disproportionate excess of 0 vs. A in a consecutive series of 191 coronary artery bypass candidates apparently conflict with epidemiological data indicating a higher risk of achieving CHD in A than 0. The conflict may be solved by suggesting a) that the "thrombotic proneness" in A compared with 0 causes a poorer prognosis in CHD among the former, leaving a disproportionate excess of 0 among longterm CHD survivors, and b) that AB0-related factors have had an insignificant, independent impact on the evolution of preclinical coronary artery disease in our 71 men with latent CHD.

ABO Blood-Group System↗