Clinical Manifestations Of Bleeding Research Articles

PECULIARITIES OF DIAGNOSTICS AND THERAPEUTIC TACTICS IN BLEEDING IN THE EARLY POSTOPERATIVE PERIOD AFTER BARIATRIC SURGERIES

Methods. We retrospectively analyzed the results of diagnostics and treatment of 14 patients who had internal bleeding in the early postoperative period after bariatric surgeries. The following parameters were studied: age and sex of the patients, average body mass index, comorbidities, volume of the performed bariatric surgery, type of internal bleeding (intraluminal/intra-abdominal), technical peculiarities of each operation, average postoperative bed-day, efficiency of instrumental diagnostic methods, types and methods of hemostasis. Results. Clinical manifestations of bleeding in all patients operated on for morbid obesity were detected on 1-2 postoperative days. The main symptoms: weakness and dizziness - in 12 cases (86%), BP decrease up to 90±60 mmHg. - in 4 (28%), tachycardia (HR 100 and more beats per minute) - in 100% of cases. Development of melena and vomiting with blood admixture was noted in 6 (42%) cases, blood flow by drainage - in 8 (58%). In 4 patients (28%) the development of bleeding was preceded by BP increase up to 180-200/100-110 mmHg. In all 14 patients hemoglobin level decreased: in 6 (42%) not lower than 100g/L (but more than 20% of the initial hemoglobin level), and in 8 (58%) - below 100g/L. Esophagogastroduodenoscopy (EGDS) was used to diagnose bleeding inside the lumen of the stomach, intestine, anastomosis, and computerized tomography (CT) of the abdominal cavity with intravenous and oral contrast for intra-abdominal bleeding. In 6 patients with intra-abdominal bleeding revision laparoscopy was performed as an emergency procedure. When the source of bleeding from the stapler line was revealed, the bleeding zone was additionally sutured with Vicryl 3\0 thread using separate knotted sutures. In case of bleeding from the trocar wound, hemostasis was performed with the help of Bersi needle using 1/0 capron thread.No lethal outcomes were observed. All patients were discharged in satisfactory condition. The average bed-day due to the complication increased on average by 2-3 days. Conclusion. Effective methods of diagnostics and treatment of intraluminal bleedings are endoscopic techniques with the possibility of reliable hemostasis. In case of intra-abdominal bleeding relaparoscopy with stitching or clipping of the bleeding source is shown.

Efficacy and Safety of Avatrombopag in the Treatment of Chemotherapy-Induced Thrombocytopenia in Children with Acute Lymphoblastic Leukemia: A Retrospective Study

Introduction: Chemotherapy-induced thrombocytopenia (CIT) is one of the most common complications during cancer treatment, increasing the risk of bleeding, medical costs, and potentially affecting treatment outcomes by necessitating chemotherapy dose reductions or delays. Avatrombopag, a thrombopoietin receptor agonist, has been approved as the first-line drug for the treatment of thrombocytopenia in chronic liver diseases. Its efficacy and safety was demonstrated in disorders like aplastic anemia and primary immune thrombocytopenia. However, the research regarding its use in children with CIT is lack. Objective: This retrospective study aimed to analyze the efficacy and safety of avatrombopag in the treatment of chemotherapy-induced thrombocytopenia in children with acute lymphoblastic leukemia, providing a potential new therapeutic option for managing CIT in pediatric cancer patients. Methods: The data of experimental group were collected from 30 pediatric patients with acute lymphoblastic leukemia and CIT who received avatrombopag treatment in the pediatric ward of Southern Hospital from August 2022 to July 2023. Additional data from 250 pediatric patients with acute lymphoblastic leukemia and CIT who did not receive avatrombopag treatment at Southern Hospital from January 2020 to July 2023 were collected as the control group. A propensity score matching method was employed to analyze the two patient groups, resulting in a successful matching of 30 pairs (CAT 21 pairs, CAT+9 pairs). The collected clinical data included age, gender, height, weight, risk stratification, infection status, platelet count changes during each chemotherapy cycle, platelet transfusion frequency, clinical manifestations of bleeding, avatrombopag usage, and complications. All patients were treated with the 2020-CCCG-ALL chemotherapy regimen,diagnosed with CIT,under 18 years of age, and excluded if they had concomitant hematological disorders, chronic liver disease, cardiovascular diseases, severe arterial or venous thrombosis, or splenomegaly. The initial avatrombopag dose was 10mg daily for patients weighing less than 30kg and 20mg daily for patients weighing 30kg or more. Avatrombopag was stopped when PLT≥100×10 9/L or an increase in PLT by ≥50×10 9/L compared to pre-treatment levels. Results: Comparison between the experimental and control groups revealed that the experimental group had a shorter duration of grade 3 or higher thrombocytopenia (3.36±1.77 days vs. 4.7±2.46 days, P=0.019) and a shorter time to platelet recovery (7.9±2.63 days vs. 9.76±2.77 days, P=0.01). Subgroup analysis within the CAT and CAT+ chemotherapy phases indicated that significant platelet value differences (P<0.05) were observed between the two groups at the start of platelet decline (day 10±2) [PLT: (268.13±216.52) ×10 9/L vs. (152.13±89.58) ×10 9/L] and during platelet nadir (day 14±2) [PLT: (435.97±285.83) ×10 9/L vs. (285.07±148.67) ×10 9/L]. Additionally, a statistically significant difference (P<0.05) in PLT change from baseline to day 5 and day 7 was observed in the experimental group [46.5 (20.5, 93) ×10 9/L and 67 (15.5, 180) ×10 9/L, respectively]. During the chemotherapy cycles, only 4 patients (8.3%) experienced bleeding events, and after avatrombopag treatment, 11 patients (22.9%) did not require platelet transfusions. Four adverse events of secondary platelet increase were recorded, and no other adverse events were reported. Conclusion: Avatrombopag, as an alternative treatment for chemotherapy-induced thrombocytopenia in children with acute lymphoblastic leukemia, demonstrated the potential to promote platelet recovery and exhibited good safety. keywords:avatrombopag,Chemotherapy-induced thrombocytopenia,acute lymphoblastic leukemia

Heterozygosity in factor XIII genes and the manifestation of mild inherited factor XIII deficiency

BackgroundThe characterization of inherited mild factor XIII deficiency is more imprecise than its rare, inherited severe forms. It is known that heterozygosity at FXIII genetic loci results in mild FXIII deficiency, characterized by circulating FXIII activity levels ranging from 20% to 60%. There exists a gap in information on 1) how genetic heterozygosity renders clinical bleeding manifestations among these individuals and 2) the reversal of unexplained bleeding upon FXIII administration in mild FXIII-deficient individuals. ObjectivesTo assess the prevalence and burden of mild FXIII deficiency among the apparently healthy German-Caucasian population and correlate it with genetic heterozygosity at FXIII and fibrinogen gene loci. MethodsPeripheral blood was collected from 752 donors selected from the general population with essentially no bleeding complications to ensure asymptomatic predisposition. These were assessed for FXIII and fibrinogen activity, and FXIII and fibrinogen genes were resequenced using next-generation sequencing. For comparison, a retrospective analysis was performed on a cohort of mild inherited FXIII deficiency patients referred to us. ResultsThe prevalence of mild FXIII deficiency was high (∼0.8%) among the screened German-Caucasian population compared with its rare-severe forms. Although no new heterozygous missense variants were found, certain combinations were relatively dominant/prevalent among the mild FXIII-deficient individuals. ConclusionThis extensive, population-based quasi-experimental approach revealed that the burden of heterozygosity in FXIII and fibrinogen gene loci causes the clinical manifestation of inherited mild FXIII deficiency, resulting in ‘’unexplained bleeding’’ upon provocation.

Korelasi Jumlah Limfosit Dengan Anti Dengue IgG/IgM

Dengue hemorrhagic fever (DHF) is a disease caused by infection with the dengue virus. DHF is an acute disease using clinical manifestations of bleeding that causes trauma and leads to death. DHF is caused by one of four virus serotypes according to the genus Flavivirus, family Flaviviridae. The diagnosis of DHF cases is supported by laboratory examination of IgG and IgM antibody parameters to determine whether they are positive or negative for DHF. Dengue IgG/IgM immunological examination is one of the crucial parameters in dengue assessment. Antibodies formed in dengue are neutralizing, anti-haemagglutinin, and anti-complement antibodies to classify primary and secondary DHF infections. Cells that play a role in the specific immune system are lymphocytes. B lymphocytes are part of adaptive immunity that can produce antibodies. Normally has an absolute lymphocyte count of 1.0-4.8 x 103/µL. The purpose of this study was to determine the relationship between lymphocyte count and anti-dengue IgG/IgM. The research method used in this research is Cross Sectional with Consecutive sampling technique. The results of the study based on the Spearman correlation statistic test obtained r = 0.082 and P = 0.667 for the relationship of Absolute Lymphocytes to IgG/IgM Results. The conclusion of this study is that there is a very weak and insignificant relationship between the number of lymphocytes and anti-Dengue IgG/IgM

IX international curse of continuing formation in haemophilia and other congenital coagulopathies. The role of the Laboratory in coagulation disorders. Diagnosis of von Willebrand disease.

Von Willebrand disease (VWD) is the most frequent inherited bleeding disorder caused by quantitative or qualitative defects of von Willebrand factor (VWF). This protein far from simplicity constitutes a very complex molecular model, remaining unravelled yet many aspects of it, even though the VWF gene (VWF) was cloned already in 1985 and the structure of VWF well defined. VWD diagnosis is difficult to achieve in a significant proportion of patients due to both disease heterogeneity and limitations in existing test processes. The cornerstone of diagnosis relies on interpretation of VWF test results, the presence of clinical manifestations of bleeding, especially mucocutaneous, and (in most cases) a positive family history. However, even with a significant bleeding history, a family history may not be positive due to factors of incomplete penetrance and variable expressivity that affect genetic changes. The laboratory diagnosis of VWD can be difficult, as the disease is heterogeneous and an array of assays is required to describe the phenotype. Basic classification of quantitative (type 1 and 3) and qualitative (type 2 variants) VWD requires determination of VWF antigenic (VWF:Ag) levels and assaying of VWF ristocetin cofactor (VWF:RCo) activity. The latter is required for identifying and subtyping VWD, but the assay is poorly standardized. For that reason, novel VWF activity assays have been developed awaiting more extensive comparison data between different methodologies and requiring validation on larger patient series. The qualitative type 2 VWF deficiency can be further divided into four different subtypes (A, B, M and N) using specific assays that measure other activities or the size distribution of VWF multimers. However, frequently, it may be difficult to correctly classify the VWD phenotype, and genetic analysis is through mutation identification may provide a tool to clarify the disorder.

Abstracts from the World Federation for Laser Dentistry—Asia Pacific Division Virtual Conference July 16–17, 2021

Abstracts from the World Federation for Laser Dentistry—Asia Pacific Division Virtual Conference July 16–17, 2021

How I treat immune thrombocytopenia – a global view

How I treat immune thrombocytopenia – a global view

UPAYA PENCEGAHAN DBD DENGAN GERAKAN SATU RUMAH SATU JUMANTIK DALAM MEWUJUDKAN MASYARAKAT PEDULI SEHAT

DHF is a health problem whose number of sufferers continues to increase and the spread is wider.DHF is an acute disease with clinical manifestations of bleeding that can lead to shock and lead to death. Various efforts have been carried out by the government such as the eradication of plural larvae through fogging, mass abasiasi movements, but this has not been optimal enough. Efforts to eradicate DHF can succeed if there is an active role from the entire community. To deal with dengue cases and their prevention special assistance is needed to work periodically in the environment. The community-run PSN program was then monitored by a larva (jumantik) observer to run effectively. The tasks of the jumantik include ensuring the existence of Aedes Aegepty mosquito larvae. A place that is suspected of being a breeding ground for mosquito larvae such as water reservoirs or clear puddles is not grounded, because the Aedes mosquito aygepty likes to lay eggs and lay their eggs in puddles that are clean and not directly grounded. In addition, jumantik also educates and helps implement 3M Plus in the home environment. The results of this service were obtained by jumantik cadres who were in charge of supervising the mosquito larvae in the house so that the dengue cases in Jambi City could be eradicated.

Advances in coagulation compensation mechanisms of patients with immune thrombocytopenia

Immune thrombocytopenia (ITP) is an acquired autoimmune hemorrhagic disease involving increased platelet destruction and insufficient platelet production, leading to thrombocytopenia, with or without clinical manifestations of bleeding. Although thrombocytopenia may cause coagulation dysfunction and hemorrhagic complications, the bleeding severity is not certainly proportional to the number of platelets. In some cases, there is no significant bleeding symptom even the platelet count is extremely low, which suggests there are additional compensatory mechanisms to control or ameliorate the bleeding complications in ITP patients. The present article aims to review recent investigations on the compensatory mechanisms of coagulation function in the patients with ITP. Key words: Thrombocytopenia; Microsomes; Phosphatidylserines; Thromboplastin; Platelet membrane glycoproteins; Thrombin; Interleukin-6

Iron Deficiency Anemia, a Rare and Potentially Underestimated Cause of Thrombocytopenia and a Differential Diagnosis of Immune Thrombocytopenia (ITP): Results from a Retrospective Case-Controlled Study

IntroductionIron deficiency anemia (IDA) is the most common cause of anemia worldwide. It is classically associated with a normal or moderately elevated platelet count, but exceptionally, iron deficiency can lead to thrombocytopenia. Distinguishing IDA-related to mucosal bleeding in the setting of immune thrombocytopenia (ITP) from a true iron deficiency-related thrombocytopenia can be challenging and may lead to misdiagnosis and inappropriate treatment.The 2 main objectives of this study were: 1) To better describe the clinical and biological baseline characteristics of clearly defined cases of iron deficiency-induced thrombocytopenia (IDIT) and 2) To identify some features that could be helpful for distinguishing IDIT and ITP.Material and methodWe performed a retrospective multicentre case-controlled study throughout the network of the French national referral center for adult' immune cytopenias. Inclusion criteria were the following: thrombocytopenia <100 x 109/L related to an IDA defined by an hemoglobin level <120 g/L and a ferritin level <30 μg/L and platelet recovery without any other treatment than iron ± transfusion of packed RBCs. Patients with pre-existing thrombocytopenia and/or other potential cause of thrombocytopenia or anemia were excluded as well as patients with a possible diagnosis of IDIT who received corticosteroids ± intravenous immunoglobulin for a putative diagnosis of ITP. Clinical and biological characteristics of the patients (i.e, the “cases”) were analyzed and each case was compared to 3 newly diagnosed ITP patients (control group) matched on age (+/- 5 years) and gender.ResultsBetween February 2000 and May 2018, eight (7 women, 87.5%) patients from 4 participating centers fulfilling the inclusion criteria were included. Median age at IDIT diagnosis was 43.5 years (Range: 16-72) years. Median hemoglobin level at diagnosis was 47 (range: 21 - 71) g/L, with a mean corpuscular volume (MCV) of 64 (54 - 75) fl, and a median platelet count of 30.5 (21 - 80 x 109/L). None of the patients but one had clinical bleeding manifestations such as purpura or mucosal bleeding. The platelet count got back to a normal level within a median of 6 (4 - 39) days after iron supplementation and/or transfusion in all 8 patients. When compared with these 8 cases, ITP controls had a significantly lower platelet count (median platelet count of respectively 7 [range 4 - 59] vs 30.5 [21 - 80] x 109/L , p = 0.002), and more bleeding manifestations (19/24 (79%) versus 1/8 (12.5%) respectively ,p = 0.001). On Day 7 after diagnosis, the median platelet count (after iron deficiency treatment or ITP treatment among the cases was 337 (113-1000 x 109/)L versus only 72 (13-212 x 109/L for the 24 controls (p = 0.001).ConclusionIn case of profound IDA, and non-severe (platelet count > 20 x 109/L) and generally asymptomatic associated-thrombocytopenia may occur and must not be misdiagnosed and treated as an ITP with secondary IDA. Iron supplementation (either intravenously or orally) allows a quick and complete recovery of the platelet count with sometimes a transient thrombocytosis and may avoid the inappropriate use of steroids. Why profound iron deficiency can seldom lead to thrombocytopenia whereas mild iron deficiency often leads to thrombocytosis needs further investigation. DisclosuresNo relevant conflicts of interest to declare.

Argininosuccinate synthase 1 (ASS1): A marker of unclassified hepatocellular adenoma and high bleeding risk.

ASS1 + HCA combined with a typical hematoxylin and eosin stain aspect defined a new HCA subgroup at a high risk of bleeding. (Hepatology 2017;66:2016-2028).

An investigation of a mass incident of bromadiolone poisoning

Objective: To investigate a mass incident of bromadiolone poisoning and analyze related clinical data. Methods: An investigation was performed for a mass incident of bromadiolone poisoning in a place in Shandong, China in December 2015, and related clinical data were analyzed and summarized. Results: This incident was a mass incident of bromadiolone poisoning caused by spreading poison. The poisoned patients had major clinical manifestations of bleeding and coagulation disorder and all of them were cured after comprehensive rescue, especially after intravenous drip of vitamin K1. Conclusion: Bromadiolone poisoning can cause severe visceral hemorrhage and coagulation disorder, and intravenous drip of vitamin K1 has a good therapeutic effect.

The causes of recurrent ulcerative gastroduodenal bleeding

To explore microcirculatory changes within the first 48 hours after admission, to compare them with clinical manifestations of bleeding and to define the dependence of recurrent bleeding from the therapy. The study included 108 patients with ulcerative gastroduodenal bleeding who were treated at the Clinical Hospital #71 for the period 2012-2014. There were 80 (74.1%) men and 28 (25.9%) women. Age ranged 20-87 years (mean 54.4±16.8 years). Patients younger than 45 years were predominant (33.4%). J. Forrest classification (1974) was used in endoscopic characterization of bleeding. Roccal Prognostic Scale for gastroduodenal bleeding was applied in all patients at admission to assess the risk of possible recurrence. Patients were divided into 2 groups. Group 1 included 53 (49.1%) patients without recurrent bleeding; group 2-55 (50.1%) patients who had recurrent bleeding within the first two days of treatment. Investigation of microcirculation showed the role of vegetative component including blood circulation centralization, blood flow slowing, blood cells redistribution providing sufficient blood oxygenation. By the end of the first day we observed pronounced hemodilution, decreased blood oxygenation, blood flow restructuring with its acceleration above 1 ml/s, violation of tissue oxygenation, signs of hypovolemia. These changes were significantly different from group 2 and associated with circulatory decentralization with possible pulmonary microcirculation disturbances and interstitial edema. This processes contribute to disruption of tissue oxygenation. We assume that recurrent bleeding in group 2 was caused by fluid therapy in larger volumes than it was necessary in this clinical situation. Infusion therapy should be significantly reduced for the debut of gastroduodenal ulcerative bleeding. Sedative therapy is advisable to reduce the influence of central nervous system.

Phototherapy in Hyperbilirubinemic Neonates; Does it Affect Platelet Count?

&lt;p&gt;&lt;strong&gt;Objective:&lt;/strong&gt;&lt;strong&gt;  &lt;/strong&gt;To determine the effect of phototherapy on platelet count in neonates having unconjugated hyperbilirubinemia.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Patients and Methods:&lt;/strong&gt;&lt;strong&gt;  &lt;/strong&gt;This cross sectional study was conducted in the Neonatology unit of a Hospital, Faisalabad during a period of nine months i.e., January to September 2015. All the neonates having unconjugated hyperbilirubinemia and requiring phototherapy with normal initial platelet count were included in the study. Platelet count was evaluated before the start of phototherapy and then during phototherapy at 24 hrs, 48 hrs and 72 hrs interval. Platelet counts were compared after 24, 48 and 72 hours of phototherapy. P value &amp;lt; 0.05 was considered significant.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results:&lt;/strong&gt;&lt;strong&gt;  &lt;/strong&gt;Total 150 newborns having unconjugated hyperbilirubinemia were included with mean age of 3.95 ± 1.71days. After 24 hours of phototherapy, about 8.1% of the patients were having severe thrombocytopenia (platelet count &amp;lt; 50 ×10&lt;sup&gt;9&lt;/sup&gt;/L); this percentage rose to 18.4% after 48 hours and 33.3% after 72 hours of phototherapy. None of the patients developed clinical manifestation of bleeding. The number of patients with normal platelet count (1,50 × 10&lt;sup&gt;9&lt;/sup&gt;/L – 400 × 10&lt;sup&gt;9&lt;/sup&gt;/L) after 24 hours of continuous phototherapy was 50%, gradually reducing  to 38% after 48 hours and only 33% after 72 hours of completion of phototherapy(p value &amp;lt; 0.05).&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusion:&lt;/strong&gt;&lt;strong&gt;  &lt;/strong&gt;Hyperbilirubinemic neonates undergoing phototherapy had a fall in platelet count without any clinical manifestation of bleeding.&lt;/p&gt;&lt;p&gt; &lt;/p&gt;

Comparison of dexamethasone and anti-D immune globulin for immune thrombocytopenia purpura in children

Different therapeutic options in children with immune thrombocytopenic purpura include observation alone, periodic treatment with corticosteroids, intravenous immunoglobulin (IVIG) or anti-D, chronic administration of immunosuppressive agents, and splenectomy.Preference of the type of therapy depends on the degree of thrombocytopenia and clinical bleeding manifestations. Dexamethasone is safe but its side effects are the main disadvantages for its usage. Anti-D is more expensive than dexamethason but the side effect is rare and not dangerous and response to treatment is assessed in approximately 3 days after infusion.

Rare Congenital Coagulation Factor Deficiencies: Clinical Manifestations

The clinical symptoms of rare bleeding disorders (RBDs) range from mucosal to life-threatening haemorrhages or functionally restricting haemorrhages. Congenital coagulation factor (F) deficiencies, VII, FX, FXIII, fibrinogen deficiencies and dysfibrinogenaemias, represent 3‒5% of all inherited coagulation factor deficiencies. Due to this low prevalence and the variability of bleeding manifestations, the clinical features of these RBDs are not well characterised. For this reason we conducted a retrospective multicentre national survey using patient records to characterize clinical bleeding manifestations in patients with congenital isolated/combined coagulation factor deficiencies or with congenital abnormalities of fibrinogen. Fifteen departments (11 haematology and four paediatric departments) across Algeria took part in the study, and data from 234 patients were analyzed. The majority of patients (n=164; 70.1%) had isolated congenital coagulation factor deficiencies, the most common of which was FVII deficiency (n=101; 43.2%). Only 221 patients (9.0%) had combined congenital coagulation deficiencies, the most common of which was FVIII/V deficiency (n=13; 5.6%). Congenital fibrinogen abnormalities were reported in 49 patients (20.9%). Overall, there were 327 bleeding episodes in 163 patients. The majority of bleeding episodes (n=195, 59.6%) were in patients with an isolated factor deficiency, the highest incidence of which was recorded for FVII deficient patients (95 bleeds; mean 1.7 bleeds/patient). Our results support existing descriptions in the literature and may help to target resourcing and plan treatment strategies for those patients at most risk of bleeding.

Bleeding and Thrombosis In 90 Iranian Patients with Inherited Afibrinogenemia.

AbstractAbstract 3667 Background:Afibrinogenemia is a rare coagulation disorder transmitted as an autosomal recessive trait. In Iran, where consanguineous marriages are common coagulation disorders occur approximately ten times more frequently than in western countries. Afibrinogenemia is characterized by abnormal thrombin time, prothrombin time, activated partial thromboplastin time and absent plasma fibrinogen levels. We investigated the clinical manifestations of bleeding and thrombosis in a series of patients with Afibrinogenemia in Iran. To our knowledge this is the largest series ever reported. Patients & Methods:All patients in our cohort were diagnosed and registered in the Iranian National Registry of Inherited Bleeding Disorders. Every patient with an inherited blood disorder is reported to this registry because it is the only source of free treatment for bleeding disorders in the country. Afibrinogenemia has been tracked in Iran since 1965. Diagnoses in all patients was confirmed when plasma fibrinogen levels were absent both as functional activity (the Clauss method) and using the antigen level (electroimmunoassay). We retrospectively evaluated data from all 90 Afibrinogenemia patients available in the registry. Results:There were 46 males and 44 females in our cohort. Ages ranged from 1 to 71 years. 82% of patients were born from consanguineous marriages with asymptomatic parents. The most frequent symptoms in our patients were umbilical cord bleeding (87%), hematomas (74%) epistaxis (70%) and hemarthrosis (52%). Central nervous system bleeding was relatively rare (2.2%). In women of reproductive age, menorrhagia occurred in 75% of cases. Recurrent spontaneous abortions (more than 2) were seen in 28% of women (range 2 to 6). With prophylactic treatment (cryoprecipitate and limited fibrinogen) two (16.6%) of our patients with recurrent spontaneous abortion out of the 12 treated were able to carry pregnancies to term. A total of 7 patients in our population developed arterial or venous thromboses (3 males and 4 females). Four cases of the thromboses observed occurred after infusion of fibrinogen concentrate. Three episodes of spontaneous thrombosis were observed including a case of mesenteric thrombosis. Discussion:Afibrinogenemia is associated with severe and life threatening bleeding symptoms. Treatment can help women afflicted with this disease carry pregnancies to term. As reported elsewhere, thrombotic complications both spontaneous and acquired from treatment were also seen in our population. Disclosures:No relevant conflicts of interest to declare.

The Usefulness of Computerized Scoring System for Assessing the Severity and Outcome of Therapy in Patients with Dengue Infection

Introduction: The clinical manifestations of dengue infection range from asymptomatic infection, undifferentiated fever, an influenza-like symptom as dengue fever (DF) to a severe, sometimes fatal disease characterized by hemorrhage and shock known as dengue hemorrhagic fever (DHF). In mild degrees of DHF (WHO grades I and II), the changes in the vital signs are minimal and transient while the severe degrees (WHO grades III and IV), also known as dengue shock syndrome (DSS), the blood pressure ranges from narrowing pulse pressure (<20 mmHg) with a characteristically elevated diastolic pressure (e.g. 100/90 mmHg) to profound shock. Bleeding diathesis is caused by vasculopathy, thrombocytopenia and coagulopathy. Vasculopathy resulting in plasma leakage is significantly found during the end of febrile stage and the toxic stage when an abrupt fall to normal or subnormal temperature occurs. The evidence of plasma leakage is manifested by hemoconcentration, hypoproteinemia, pleural effusion, ascites and shock. The toxic stage lasts 24–48 hours followed by the uneventful convalescent stage in the majority of patients. Therapy of DHF is wholly symptomatic and aims at controlling the clinical manifestations of bleeding and shock. The rate and volume of fluid replacement should be adjusted according to the rate of plasma leakage as guided by hematocrit, vital signs and urine output. Frequent monitoring of vital signs, hematocrit and urine output is necessary for evaluating the outcome of therapy. Patients who do not receive the appropriate treatment usually die within 12–24 hours after the occurrence of shock. Prolonged shock is often complicated with metabolic acidosis that may precipitate disseminated intravascular coagulation (DIC) or enhance the ongoing DIC to aggravate massive bleeding. The close monitoring of clinical manifestations and results of laboratory tests by the attending physicians and nurses is essential for favorable outcome.Methods: A computerized program of scoring system for assessing the clinical manifestations and outcome of management in patients with dengue infection, was created. It was written by Microsoft Access. Sixteen items involving vital signs of pulse rate, blood pressure, duration to achieve the pulse pressure ≥ 20 mmHg, urine output; number of bleeding site; amount of required crystalloid, colloid, packed red cells and platelet concentrate; manifestations of hepatic failure, encephalopathy, respiratory distress syndrome, reshock or requiring inotropic drugs; as well as the requirement of central line application, ventilator or dialysis, were included. The scores of 3 to 10 were given to each item with a total score of 100. The scores in each item was also classified by the severity of clinical manifestations according to the patient's age. The higher scores were given to a more severe manifestation. The total scores would be automatically shown on the computerized screen when the clinical data input was added.Results: The scoring system was retrospectively and prospectively applied to children with dengue infection whose ages ranged from 5–15 years. All patients had positive dengue-specific IgM and IgG antibodies in the acute and convalescent sera determined by capture ELISA technique. The results revealed that the scores increased accordingly with the severity of the manifestations (DF 0–10, DHF grade I 5–15, grade II 10–25, Grade III 20–50, grade IV 40–75 and DSS with reshock or uncontrolled bleeding 50–100). The case-fatality was found in patients with higher scores of 75–100.Conclusion: The computerized scoring system for assessing the severity of patients with dengue infection is helpful in the clinical practice especially for the management of high risk patients with severe degree or DSS.

Thromboelastography in children with coagulation factor deficiencies

Hemophilia is traditionally classified according to the levels of the deficient coagulation factor as Severe (<1%), Moderate (1-5%) or Mild (>5%). However, it is well known that the factor activity does not necessarily correspond to the clinical bleeding manifestations. As prophylactic therapy is the best method of prevention of serious complications such as hemophilic arthropathy, a test that may predict the bleeding pattern would be extremely beneficial. Thromboelastography (TEG) uses whole blood to determine clot formation characteristics, such as initiation, propagation as well as strength of the clot, and is now being extensively studied in bleeding and thrombophilia. This study attempted to determine the TEG characteristics in 47 children with moderate hemophilia (MH) and severe hemophilia with (SHI) and without inhibitors (SH) and tried to retrospectively correlate them to the clinical bleeding patterns. TEG showed evidence of faster and better clot formation, as evidenced by a higher maximum thrombin/fibrin generation, in those with mild bleeding manifestations compared to those with severe bleeding tendency, in addition to the expected prolongation in time to formation of clot related to factor deficiency. This may be a potentially useful tool to evaluate the bleeding tendency and determine need for prophylaxis in children with hemophilia.

Can blood flow assays help to identify clinically relevant differences in von Willebrand factor functionality in von Willebrand disease types 1-3?

Can blood flow assays help to identify clinically relevant differences in von Willebrand factor functionality in von Willebrand disease types 1-3?