[Diagnostic approach to von Willebrand's disease in childhood]. / Dijagnosticki pristup von Willebrandovoj bolesti u decijem uzrastu.

Von Willebrand's disease seems to be the most common hereditary bleeding disorder in children. Every form of this disease is based on quantitative or qualitative disorder of von Willebrand's factor influencing adhesion of thrombocytes (primary hemostasis) and stabilization FVIII:C in circulation (secondary hemostasis). We present contemporary knowledge on epidemiologic investigations, molecular biology, classification and rational diagnostic approach to von Willebrand's disease in children. We also report on evaluating laboratory tests' reliability in 51 examined children with von Willebrand's disease. It can be concluded that most laboratory tests are highly specific and significant for diagnosis. Determination of vWF activity has optimal value for establishing laboratory diagnosis of von Willebrand's disease; RIPA and multimer analysis are valuable for subtype classification.

[Lupus anticoagulant in pregnancy--first experiences in detection and therapy]. / Lupusni antikoagulans u trudnoci--prva iskustva u dijagnostici i terapiji.

We examined 62 women with three or more recurrent adverse pregnancy outcomes where we excluded the other well known causes of this state. We detected lupus anticoagulant (LAC) in three (5%), with the use of set of coagulation tests: activated partial thromboplastin time (APTT), APTT ratio, kaolin clotting time ratio (KCT), APTT of the 50:50 mixture of the patients and control plasmas, APTT ratio of the 50:50 mixture, phospholipid correction test and heat stability test. We excluded connective tissue disorders. Therapy was started between 12th and 14th gestational week. Efficacy was monitored in intervals of 2-6 weeks with following tests: APTT ratio, KCT and plateled count. Patient A, with strong LAC activity and thrombocytophenia, reacted weakly to therapy with Pronison 40 mg/day and Aspirin 80 mg/day. The activity of LAC was slightly diminished but was present all the time. Patient expressed hypertension and gestational diabetes mellitus. The outcome of pregnancy was adverse and placenta had typical pathological changes. Patient B, with moderate LAC activity, reacted quickly and completly on therapy with Pronison 20-40 mg/day and Aspirin 80 mg/day. The course of pregnancy was regular. The outcome was successful and placenta had no pathological changes. Patient C, with mild LAC activity, was treated only with Aspirin 80 mg/day. LAC activity rapidly disappeared, the course of pregnancy was regular and outcome was successful. On the basis of the first results we concluded that applied set of tests was sensitive for LAC of different intensity and that dissapeparance of LAC activity with the use of therapy anticipates successful pregnancy outcome.

Primary wound closure in haemophiliacs undergoing dental extractions.

215 teeth were extracted in 62 haemophiliacs following a single infusion of factor VIII supplemented with antifibrinolytics. Tooth sockets were packed with Sorbacel gauze soaked with an antifibrinolytic. 1 group was sutured and the other left open. Primary closure of the extraction wound did not show direct effect on haemostasis. However, the size of the clot showed a statistically significant difference between the primary closure and open wound groups. The authors conclude that primary closure of the extraction wound protects the blood clot, makes the postoperative period more comfortable for patients and may subsequently decrease the risk of postoperative bleeding.

[Comparative study of concentrated blood derivatives of factor VIII]. / Uporedna ispitivanja koncentrovanih krvnih derivata F VIII.

The work presents results of the investigations of blood derivatives--F VIII concentrates: commercial cryoprecipitate, concentrate of intermediary purity and derivatives of high purity: Kriobulin--Immuno, Octobulin--Landerlan, Profilate--Alfa, Factor VIII--Behring, Hemofil--Hyland, Factorate--Armour Pharma, AHF--Kaote Cutter. The following parameters were investigated: VIII: C, VIIIR: Ag, total protein, protein electrophoresis, IgG, IgA and IgM immunoglobulins and anti-A and anti-B isoagglutinins. All derivatives except cyroprecipitate have considerably higher VIIIR: RAg value compared with VIII: C, which indicated inactivation of labile VIII: C component during concentrate preparation. Specific activity varied depending on purity of preparations, but ranged from 1,72 to 22. High isoagglutinin titer of anti-A was noted in preparations of high purity, as well as the presence of immunoglobulins. Despite considerable differences in vitro, all concentrated derivatives F VIII have similar immediate clinical effect and recovery from 0,87 to 1,36. All results indicate that new ways of derivative F VIII purification should be found with lower degree of contamination of other plasma proteins and less risk of hepatitis virus transmission. When certain indications are recognized, cryoprecipitate produced in our country in all blood transfusion services should be used.

[Physiologic values of coagulation factors in newborn infants]. / Fizioloske vrednosti cinilaca koagulacije u novorodene dece.

Hemorrhagic tendencies are frequently seen in the neonatal period. From the practical point of view, recognition of physiologic deficiencies of coagulation factors and hemostasis is essential, for the differential diagnosis of hemorrhagic syndromes and adequate treatment. Values of coagulation factors in the newborn and comparison of our results and the results of other authors are presented. It seems that there are no essential changes in hemostasis of term infant, but many coagulation factors exhibit deficient clotting activity as like vitamin K-dependent factors (II, VII, IX, X). Values of fibrinogen, F. XI, XII, XIII are decreased. Those factors which physiologically low in the term infant reach normal adult levels in a few weeks, with the exception of factors IX and XI which may require several months to reach normal levels. In the same time authors presented the results of coagulation factors in mothers during delivery and term infants, to explain the nature of the disorders. For the investigation of these physiological defects, screening tests can be used.

[Chronic granulomatous disease (in a 2-month-old infant)]. / Hronicna granulomatozna bolest (u odojceta od 2 meseca).

The paper describes a severe form of chronic granulomatous disease with onset in the neonatal period; the case presented is a 2-month-old male infant, coming from a family in which hid older brother died with signs of the same illness. The disease is rare. This is the second report in our literature. The clinical picture showing various severity confirms the heterogenity of the disease and difference in the gene expressivity. Further investigation involving the function of granulocytes in children with recurrent infections might help us to reveal this disease characterized by disfunction of granulocytes, more frequently.

[Transfusion of leukocytes and platelets in the treatment of acute leukemia]. / Transfuzija leukocita i trombocita u lecenju akutnih leukemija.

Supportive therapy of concentrated leukocytes and platelets has no obvious significance. In the paper authors presented the methods of separation of concentrated leukocytes and platelets, investigation of number and functional abilities of preserved leukocytes and platelets as well as the monitoring of the transfusion effect in the patients with acute myeloblastic leukemia. Special attention was paid to the separation of blood cells by the cell centrifuge with continuous flow. Side effects after leukocytes and platelet transfusion was monitored -- occurence of the sensibilization to the HL-A antigens. For the detection of HL-A antibodies two-stage microlymphocytotoxic test was used for the investigation of patient's serum; for the application of leukocyte and platelet transfusion we used cross-match test between donor's lymphocytes and patient's serum in progressive dilution. In the paper authors discussed the correlation between the appearance of HL-A antibodies and the number of given transfusions.

[Wiskott-Aldrich syndrome]. / Sindrom Wiscott- Aldrich.

Within the group of diseases of immunodeficiency Wiscott-Aldrich syndrome can be excepted as one with well definical picture. Here presented are two patients with allness onset in the earliest childhood. Their chinical picture was a typical one showing often infections, thrombocytopenia and later appearance of eczema. Beside the dicreased number of the thrombocytes both patients had eosynophilia, changed immunoglobulines and lack of isohemogglutinin. One of them also showed low response to phytohemagglutinin and low values of the T and B rosettes. One of the patients lived until four years of age while the other one is aged five now and is in relatively good condition.

[Diagnosis of von Willebrand's disease]. / Danasnje mogucnosti dijagnostike von Willebrand-ovog oboljenja.

Diagnosis and differential diagnosis of von Willebrand's disease was a special problem. Criteria up to date: prolonged bleeding time, reduced platelet adhesiveness, decreased F. VIII coagulant activity, as well as a particularly behaviour after the infusion of F. VIII, were not sufficient to differentiatie this disease from other congenital disorder of F. VIII. Recent investigations, introducing the immunological methods for determination of F. VIII--related antigen, as well as the investigation of ristocetin induced platelet aggregation, give the new approach in the diagnosis of von Willebrand's disease. Authors presented preliminary results of investigations of F. VIII--related antigen in the patients with von Willebrand's disease, as well as the results of investigation of the patients with hemophilia A and normal subjects, as a control group.

[Immunoelectrophoretic method for the determination of factor VIII-related antigens]. / Imunoelektroforetska metoda u odredivanju F. VIII srodnog antigena.

Immunological examination of F VIII related antigen gave some new information on the nature of congenital defects of F VIII, in haemophilia A and von Willenrand's disease. Besides classic method in diagnostics of von Willebrand's disease, the determination of F VIII related antigen can be used as a diagnostic criterium in distinguishing von Willebrand's disease from some mild forms of haemophilis, as well as in detection of haemophilia carrier. In addition, the study on the relationship of immunological value of F VIII related antigen and biological activity of F VIII offers more data on the possibility of detection of so-called "hypercoagulability" and states proceeding thrombosis. The method for determination of F VIII related antigen (Rocket electrophoresis--Laurel) as well as the values of F VIII in health persons of our population is described in this paper.

[Substitution therapy in the treatment of acute leukoses]. / Supstituciona therapija u lecenju akutnih leukoza

In the treatment of acute leukoses, from the first onset of the disease till the occurrence of the first remission, as well as in the course of intesive hemiotherapy, the substitution therapy by blood derivatives has the great importance. Anaemia, granulocytopenia, hypoproteinaemia and hemorrhagic syndroms, caused by thrombocytopenia, liver diseases and disseminated intravascular coagulation, are the most frequent disorders requiring the substitution therapy. Authors presented the advantages of the substitution therapy by blood derivatives upon the whole blood.

[Experiences at the Blood Transfusion Center of the Socialist Republic of Serbia in providing aimed transfusions]. / Iskustva zavoda za transfuziju krvi SRS u obezbedenju ciljanih transfuzija

The development of modern medicine causes the more and more needs of blood in providing the complete health care of the patients. The increased need can be satisfied only by the rational use of blood on the principle of selective transfusions. In this work our present possibilities are given, in the separations of certain blood components, their good and bad effects, as well as the indications for use in some pathological states.