scholarly journals Defibrination Syndrome Developed after Replacement Therapy with PPSB in a Case of Hemophilia B.

1977 ◽  
Author(s):  
M. Kazama ◽  
H. Iwakura ◽  
M. Nakajima ◽  
M. Abe ◽  
T. Abe
Keyword(s):  
Bleeding Complication ◽  
Severe Pain ◽  
Coagulation Factors ◽  
Massive Bleeding ◽  
Hemophilia B ◽  
Valgus Osteotomy ◽  
The Third ◽  
Coagulation Tests ◽  
Fresh Plasma ◽  
Large Hematoma

A case of Hemophilia B, 20y., had valgus osteotomy of the left femoral neck. The preliminary replacement with PPSB was revealed not to introduce inhibitor and no other abnormal enzyme activity was found except for the slight disturbance of liver function. The operation was gotten ready with injection of 10 vials PPSB and performed smoothly in 2 hours with 340ml of blood loss. PPSB administration was scheduled in a daily dosis of 20 vials divided in twice. But at the third postoperative day, large hematoma concreted in the femoral region with severe pain and was followed by the massive bleeding from the wound. Coagulation tests revealed decreased platelet count, prolonged.PTT and prothrombin time, decreased fibrinogen and increased FDP. The concentration of F.IX was not lifted so high as expected after the injection of 10 vials of PPSB, which, together with the above mentioned changes of coagulation factors, suggested the developement of defibrination syndrome.The replacement of PPSB was continued together with 10,000 U of heparin and 800ml of fresh plasma per day. At the 7th postoperative day, the coagulation findings of defibrination syndrome were gradually improved, bleeding decreased and F. IX level elevated as could be calculated. The administration of PPSB was diminished and discontinued at 29th day and of heparin at 45th day without any bleeding complication thereafter.

EPTACOG ALFA. APPLICATION EXPERIENCE

2017 ◽  
Author(s):  
И. Нехаев ◽  
А. Приходченко ◽  
С. Ломидзе ◽  
А. Сытов
Keyword(s):  
Intensive Care Unit ◽  
Malignant Tumors ◽  
Coagulation Factors ◽  
Fresh Frozen Plasma ◽  
Massive Bleeding ◽  
Threshold Concentration ◽  
Damage Area ◽  
Fresh Frozen ◽  
Hemostatic Therapy ◽  
Frozen Plasma

Введение. Несмотря на переливания свежезамороженной плазмы и тромбоцитов, часто не удается достигнуть нужной «пороговой» концентрации факторов свертывания при массивных кровотечениях. При введении рекомбинантного активированного VII фактора (rFVIIa, эптаког альфа) этот процесс может быть ускорен, происходит «тромбиновый взрыв», который обеспечивает образование стабильной фибриновой пробки. Цель исследования: оценка эффективности и безопасности применения rVIIа в онкохирургии при коагулопатических кровотечениях. Материалы и методы. Обследовано 38 пациентов, оперированных по поводу злокачественных новообразований различной локализации, находившихся на лечении в отделении реанимации и интенсивной терапии № 1 в течение 2014 года. Результаты. Клиническая эффективность rFVIIа составила 94,7% при неэффективности стандартной гемостатической терапии и исчерпанных возможностях хирургического гемостаза при коагулопатических кровотечениях у онкохирургических больных. Заключение. rFVIIа обладает селективным действием (действует в зоне повреждения), что подтверждают данные коагулограммы и тромбоэлаcтометрии. rFVIIа не утяжеляет состояния больных. Introduction. Despite the transfusion of fresh frozen plasma and platelets it is often not possible to achieve the desired «threshold» concentration of coagulation factors in case of acute massive bleeding. Administration of recombinant activated VII factor (rFVIIa, eptacog alfa) can accelerates this process; «thrombin burst» occurs that provides the formation of a stable fi brin plug. Aim: to assess the effectiveness and safety of rVIIa usage in oncosurgery at coagulopathic bleedings. Materials and methods. In intensive care unit during 2014 we examined 38 patients with malignant tumors of various locations after surgery. Results. Clinical efficacy of rFVIIa was 94,7% with ineffectiveness of standard hemostatic therapy and exhausted possibilities of surgical hemostasis with coagulopathic bleedings in oncosurgical patients. Conclusion. rFVIIa has a targeted action (acts in damage area); coagulogram and thromboelometry data prove its action. rFVII does not make patients worse.

scholarly journals Dr P. Davis, prof. Mann et al. — Fatal Nausea and Vomiting of Pregnancy. - (New-Jork Medic. Journ., 1894, 28 / VII, p. 121). Fatal nausea and vomiting in pregnant women. Dr. Terrel. - The Black vomit of Pregnancy. - (Ibid, 18 / VIII p. 212). Black vomiting in pregnant women

2020 ◽  
Vol 9 (7-8) ◽  
pp. 698-699
Author(s):  
M. Ginzburg
Keyword(s):  
Pregnant Woman ◽  
Pregnant Women ◽  
Uterine Cervix ◽  
Severe Pain ◽  
Fatty Degeneration ◽  
Nausea And Vomiting ◽  
Minor Bleeding ◽  
Slight Improvement ◽  
Severe Nausea ◽  
The Third

Dr. Davis reported three fatal cases of vomiting during pregnancy. In the first of them, the pregnant woman suffered from gastric disorders before pregnancy; her vomiting was incessant and very exhausted her. She died at 2 months of pregnancy. Before dying, she developed a petechia-shaped spotty rash. In the second case, severe nausea and vomiting occurred at 3 weeks of pregnancy with severe pain and belching, locally: prolapsus and anteflexio uteri. The usual treatment. At 14 weeks, the anteflexed uterus was infringed at the bottom behind the pubis; the correction did not reduce vomiting. The expansion of the neck was accompanied by a slight improvement. It was decided to empty the uterus, which was done without difficulty and with minor bleeding. The patient, however, died soon after. An autopsy showed that the tissue of the uterine cervix was abnormally hard (fibrous); the uterus, ovaries, and tubes were normal; the blood was thin, fatty degeneration of the heart. In the third case, the patient, in addition to nausea and vomiting, suffered from severe pain in the epigastric region and vomiting had a coffee color. Everything possible was done, but nothing helped: the patient died.

scholarly journals Advances in the Treatment of Hemophilia: Implications for Laboratory Testing

2019 ◽  
Vol 65 (2) ◽  
pp. 254-262 ◽  
Author(s):  
Armando Tripodi ◽  
Veena Chantarangkul ◽  
Cristina Novembrino ◽  
Flora Peyvandi
Keyword(s):  
Coagulation Factors ◽  
Alternative Methods ◽  
Laboratory Evaluation ◽  
One Stage ◽  
New Therapeutic Approaches ◽  
Novel Drugs ◽  
Coagulation Tests ◽  
Bypassing Agents ◽  
Long Acting ◽  
Near Future

Abstract BACKGROUND Until recently, clinical laboratories have monitored hemophilia treatment by measuring coagulation factors before/after infusion of human-derived or recombinant factors. Substantial changes are expected in the near future based on new therapeutic approaches that have been or are being developed. CONTENT Hemophilia treatment includes replacement therapy with human-derived/recombinant factors or treatment with bypassing agents for patients without or with inhibitors, respectively. Accordingly, laboratory methods for monitoring include one-stage clotting or chromogenic assays meant to measure either factor VIII/IX or global coagulation tests to measure the effect of bypassing agents. Recently, modified long-acting coagulation factors have been introduced for which discrepant results may be expected when measurement is performed with one-stage clotting or chromogenic assays. Currently, novel drugs not based on coagulation factors are under development and are being tested in clinical studies. These drugs do require new methods and therefore laboratory evaluation of hemophilia will undergo dramatic changes in the near future. SUMMARY From the analysis of the current practice and literature, we draw the following conclusions: (a) Thrombin generation or thromboelastometry are the logical candidate assays to monitor bypassing agents. (b) Considerable differences are expected when measuring modified long-acting coagulation factors, depending on whether one-stage or chromogenic assays are used. Although no definitive conclusions can presently be drawn, chromogenic assays are probably more suitable than one-stage clotting. (c) Novel drugs not based on coagulation factors such as emicizumab, fitusiran, or concizumab that are entering the market do require alternative methods that are not yet well established.

Efficiency and safety of sclerotherapy for recurrent nodular goiter

2020 ◽  
pp. 39-51
Author(s):  
G. V. Rodoman ◽  
I. R. Sumedi ◽  
N. V. Sviridenko ◽  
T. I. Shalaeva ◽  
M. M. Meloyan
Keyword(s):  
Minimally Invasive ◽  
Severe Pain ◽  
Nodular Goiter ◽  
Treatment Method ◽  
Age Group ◽  
Invasive Treatment ◽  
The Third ◽  
Invasive Method ◽  
Functional Autonomy

At present, patients with recurrent nodular goiter account for a significant portion of patients operated on for nodular goiter. At the same time, the comorbid background characteristic of this age group and the technical difficulties of the intervention on cicatricial tissues of the neck cause a high risk of complications of the operation, 3–7 times higher than with primary thyroid interventions. The aim of the study was to evaluate the effectiveness and safety of treatment of recurrent nodular goiter using an alternative minimally invasive method — sclerotherapy. The study included 30 patients previously operated on for nodular goiter. All had 4 courses of sclerotherapy, each included 5 sessions with a frequency of 1 session per week, followed by a follow-up period of 3 months. Polydocanol was used as a sclerosant. The analysis showed that sclerotherapy for recurrent nodular goiter allows all patients to reduce recurrent nodular formations, and in almost a third of cases, complete reduction of the nodes. On average, the decrease in the volume of thyroid residues was 9.6 ± 1.5 ml, and the size of nodular formations decreased by 17.2 ± 1.3 mm (3.7 times — from 23.6 ± 1.4 mm to 6.4 ± 0.7 mm, P <0.001). Nodes more than 3 cm, initially 19 %, ceased to be detected after the third course of sclerotherapy. In all cases, managed to eliminate hormonal imbalances in patients who initially had functional autonomy, as well as signs of compression of the neck organs. At the same time, sclerotherapy of nodules of the thyroid gland using polydocanol as a sclerosant is a safe minimally invasive treatment method, is not accompanied by severe pain and the risk of hypoparathyroidism and laryngeal paresis.

EFFECTS OF FRESH PLASMA OR WHOLE BLOOD TRANSFUSIONS ON PATIENTS WITH VARIOUS TYPES OF MUCOPOLYSACCHARIDOSIS

PEDIATRICS ◽  
1972 ◽  
Vol 50 (5) ◽  
pp. 688-692
Author(s):  
Anatole S. Dekaban ◽  
Kenton R. Holden ◽  
George Constantopoulos
Keyword(s):  
Molecular Weight ◽  
Weight Distribution ◽  
Whole Blood ◽  
Blood Transfusions ◽  
Molecular Weights ◽  
The Third ◽  
Clinical Observations ◽  
Before And After ◽  
Fresh Plasma

Repeated fresh plasma or whole blood transfusions were given to five patients with either Hurler, Hunter, or Sanfilippo types of mucopolysaccharidosis. Clinical observations and total 24-hour urinary AMPS and their composition and molecular weight distribution were determined before, during, and after transfusions. The two patients who received plasma transfusions showed no noticeable change in the amount of AMPS excreted; of the three patients who received whole blood transfusions, two had slightly less excretion of AMPS while the third showed no difference. The AMPS in the CSF were measured in one patient before and after blood transfusions and found to be unchanged; likewise, the determination of molecular weights in the isolated AMPS was virtually identical. In the patients studied, the transfusions caused no demonstrable difference in the patients' clinical condition.

Influence Of Elastase On Blood Coagulation Factors: Studies In Vitro, In Rats And In Göttinger Miniatur Pigs

1981 ◽  
Author(s):  
U Kasten ◽  
U Artmann ◽  
T Kaethner ◽  
H Burchardi ◽  
H Köstering
Keyword(s):  
Blood Coagulation ◽  
Coagulation Factors ◽  
Bleeding Complications ◽  
Thrombin Time ◽  
Normal Range ◽  
Blood Coagulation Factors ◽  
Acute Respiratory Insufficiency ◽  
Coagulation Tests ◽  
Antifibrinolytic Drugs

The influence of blood coagulation factors in pat. with acute respiratory insufficiency of adults, especially of the so called “pancreatitis lungs” is still unknown. In order to find out the effect of elastase, possibly activated by trypsin in pat. with acute pancreatitis, on blood coagulation factors, we performed some studies. In vitro elastase induces in plasma and blood in correlation to the dosages Enhancement of thrombingeneration in the TGT, a shortening of PTT, Thrombin time and of r- and k-time in the TEG, a loss of fibrinogen and an increase of fibrinmono-mercomplexes. In another study, elastase (960 U/ kg b.w.) was injected intravenously in rats. 30 min. later there was found a loss of fibrinogen, number of platelets, Prothrombin and a prolongation of PTT and Thrombin time and an increase of fibrinomonomercomplexes, especially in these rats, which received beside elastase Kalikreininhibitors or antifibrinolytic drugs. After repeated injections (3 times within 30 h) we found histomorpholgically thrombi as well as bleeding complications. In another study we performed (150 min) an infusion of elastase (333 U/kg b.w./h) to 9 pigs. We determined a loss of fibrinogen of platelets, of F. II, F. VII and F. XIII, a prolongation of PTT. F. VIII and F. V remained within the normal range But there was found an enhancement of Thrombin generation in the TGT, too. Compariening the results of blood coagulation tests and of histomorphological findings, elastase induced a DIC. We have to discuss their influence on ARIA and “Pancreatic lungs”.

LUPUS ANTICOAGULANT (LA) COEXISTENT WITH TRANSIENT PROTHROMBIN (FII) INHIBITOR: FTI DEFICIENCY DUE TO CLEARANCE OF THE B/MUNOCOMPLEX

1987 ◽  
Author(s):  
R Redaelli ◽  
F Baudo ◽  
B Busnach ◽  
T M Caimi ◽  
L Perrino ◽  
...  
Keyword(s):  
Survival Time ◽  
Replacement Therapy ◽  
Lupus Anticoagulant ◽  
Normal Tissue ◽  
Past History ◽  
Laboratory Data ◽  
Coagulation Factors ◽  
Precipitin Line ◽  
Coagulation Tests ◽  
Tissue Thromboplastin

23 y.o. man with acute nephritis and bleeding (epistaxis, ecchymosis) at presen-taticn. Family and personal past history negative for bleeding. Laboratory data consistent with SLE. Coagulation tests: FT Ratio (R) 1.8, aPTT R 2.4, FII:C <1%, FIIR:Ag 996, other coagulation factors normal. Tissue thromboplastin inhibition test (TTIT) R 2.8, congenital FII deficiency (696) R 1.6.1. FII survival time (Fll-ccncentrate infusion - 60 U/kg) t1/2: 9 hours.2. FII neutralizing activity (FTI:C normal plasma (NP) + buffer 5996; NP + patient plasna {PtP) 5096): absent.3. Irmunoccrplex formaticn4. FII inhibitor characterization (purified FII coupled to CNBr-activatedSepharose →PtP incubation with Fll-Sepharose→specific antiFII irrrrunoglobulins (Ig)* elution at acid pH→identification by double iimunodifftision): precipitin line with anti IgA, anti IgG2, anti k, anti 1.5. LA characterization (after FII inhibitor disappearance): TTTT on mixtures NP + PtP or N Ig in equal volumes.Diagnosis: SIE, LA (IgG); polyclonal (IgA, IgG2, k, 1) not neutralizing FII inhibitor; hypoprothrxmbinemia due to clearance of the irrrrunocorrplex.FII inhibitor was transient. Bleeding was rapidly controlled by replacement therapy. LA persits after FII inhibitor disappearance.

ANTICOAGULANT EFFECT OF INCUBATED FIBRINOGEN

1958 ◽  
Vol 36 (1) ◽  
pp. 249-259 ◽  
Author(s):  
D. C. Triantaphyllopoulos
Keyword(s):  
Ammonium Sulphate ◽  
Coagulation Factors ◽  
The Other ◽  
Anticoagulant Effect ◽  
Factor Vii ◽  
Factor V ◽  
Thrombin Time ◽  
Fresh Plasma ◽  
Native Plasma ◽  
Ammonium Sulphate Saturation

Sterile fibrinogen rendered non-clottable by incubation was mixed with fresh plasma and the thrombin time determined. An appreciable prolongation was observed. The incubated fibrinogen was then fractionally precipitated with ammonium sulphate. The material precipitated between 25 and 50% ammonium sulphate saturation, when added to freshly drawn but still unclotted blood, or native plasma, prevented its coagulation. This action could be reversed by an approximately fivefold dilution with distilled water and addition of calcium chloride and thrombin, thus excluding fibrinolysis as the cause of the anticoagulant effect. Determinations of the respective coagulation factors showed that no decrease occurred in prothrombin, factor VII, plasma thromboplastin component, and fibrinogen. On the other hand a statistically significant decrease in factor V was observed when calcium was present.

scholarly journals The Effect of 3.2% and 3.8% Sodium Citrate on Specialized Coagulation Tests

2018 ◽  
Vol 142 (8) ◽  
pp. 992-997
Author(s):  
Franz Ratzinger ◽  
Mona Lang; ◽  
Sabine Belik; ◽  
Klaus G. Schmetterer ◽  
Helmuth Haslacher ◽  
...  
Keyword(s):  
Coagulation Factors ◽  
Reference Ranges ◽  
Buffer Concentration ◽  
Citrate Buffer ◽  
Citrate Concentration ◽  
Confirmation Test ◽  
Coagulation Testing ◽  
Significant Difference ◽  
Coagulation Tests ◽  
The Difference

Context.— Coagulation testing is challenging and depends on preanalytic factors, including the citrate buffer concentration used. Objective.— To better estimate preanalytic effects of the citrate buffer concentration in use, the difference between results obtained by samples with 3.2% and 3.8% citrate was evaluated. Design.— In a prospective observational study with 76 volunteers, differences related to the citrate concentration were evaluated. For both buffer concentrations, reference range intervals were established according to the recommendations of the C28-A3 guideline published by the Clinical and Laboratory Standards Institute. Results.— In our reagent-analyzer settings, most parameters evaluated presented good comparability between citrated samples taken with 3.2% and 3.8% trisodium buffer. The ellagic acid containing activated partial thromboplastin time reagent (aPTT-FS) indicated a systemic and proportional difference between both buffer concentrations, leading to an alteration in its reference ranges. Further, a confirmation test for lupus anticoagulant assessment (Staclot LA) showed only a moderate correlation (rρ = 0.511) with a proportional deviation between both citrate concentrations. Further, a statistically significant difference was found in the diluted Russell viper venom time confirmation testing, coagulation factors V and VIII, and the protein C activity, which was found to be of minor clinical relevance. Conclusions.— With caution regarding the potential impact of the reagent-analyzer combination, our findings demonstrate the comparability of data assessed with 3.2% and 3.8% buffered citrated plasma. As an exception, the aPTT-FS and the Staclot LA assay were considerably affected by the citrate concentration used. Further studies are required to confirm our finding using different reagent-analyzer combinations.

A Novel Congenital Bleeding Disorder Related to High Plasma Heparin-Like Anticoagulant Activity.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4074-4074
Author(s):  
Zhaoyue Wang ◽  
Haiyen Yang ◽  
Xia Bai ◽  
Wei Zhang ◽  
Changgeng Ruan
Keyword(s):  
Ex Vivo ◽  
Anticoagulant Activity ◽  
Coagulation Factors ◽  
High Plasma ◽  
Bleeding Disorder ◽  
Normal Plasma ◽  
Normal Ranges ◽  
Coagulation Tests ◽  
Plasma Heparin

Abstract Heparin or heparin-like compounds present in human plasma in minute amounts. It has been reported that a very few patients with such diseases as plasma cell neoplasms, acute monoblastic leukemia and acquired immune deficincy syndrome have an increased plasma heparin-like anticoagulant activity. Recently, we found a 10-year-old girl who was physically and developmentally normal, but had recurrent episodes of prolonged bleeding and hematoma starting in her early childhood, which could be stopped by transfusion of fresh frozen plasma or prothrombin complex concentrate. The coagulation tests of her plasma were regularly repeated since she was 2 years old, and always revealed a markedly prolonged APTT (61.8–104 seconds, normal 28–40 seconds) and TT (36–50.1 seconds, normal 14–21 seconds), and a slightly prolonged PT (15.9–25 seconds, normal 11–14.5 seconds). Fibrinogen, prothrombin and other coagulation factors as well as anticoagulant and fibrinolytic systems were all normal. The results of immunologic measurements were either negative or within normal ranges. Treatment of the patient’s plasma in vitro with either protamine or heparinase could completely normalize the coagulation abnormalities, but not with normal plasma. The anticoagulant activity of her plasma corresponded to 0.2 heparin U/mL when measured by a TT assay using normal plasma as substrate and standardized with porcine heparin. Her plasma heparin concentration was 0.22 heparin U/mL when measured using a colometric assay. In ex vivo study, the abnormal coagulation tests could effectively be corrected when the patient was intravenously administed with protamine. Considering these characteristic laboratory features of the patient, we suppose it would probably represent a novel congenital bleeding disorder related to high plasma heparin-like anticoagulant activity which, to our knowledge, had not been described before.

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