Homozygous protein C deficiency is a rare hereditary coagulation disorder that occurs most often in childhood and is characterized by widespread thrombosis of capillaries and venules, abrupt onset of ecchymoses and necrosis. The hematological feature corresponds with disseminated intravascular coagulation. Protein C is a natural anticoagulant and also has important anti-inflammatory activity. For confirmation of homozygous protein C deficiency, the infant should have undetectable protein C activity and both parents should be heterozygous for protein C deficiency. We experienced a case of purpura fulminans in the newborn infant in whom we identifed homozygosity for familial protein C deficiency. Fresh frozen plasma for replacement of protein C, early debridement and full-thickness skin graft induced a remission. Administration of warfarin was used to prevent recurrence of attacks. This report emphasizes the need for early diagnosis and adequate replacement therapy in patient with purpura fulminans.
Blood Coagulation Disorders, Inherited ; Capillaries ; Debridement ; Disseminated Intravascular Coagulation ; Early Diagnosis ; Ecchymosis ; Humans ; Infant ; Infant, Newborn ; Necrosis ; Parents ; Plasma ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans* ; Purpura* ; Recurrence ; Skin ; Thrombosis ; Transplants ; Venules ; Warfarin

In general, FDP and D-dimer values have a correlation in clinical conditions associated with disseminated intravascular coagulation(DIC) or coagulation activation. However, there are some patients with discordant results who demonstrate elevated FDP and negative D-dimer results by latex agglutination assays. The incidence and possible reasons for the discordance between FDP and D-dimer results were investigated through simultaneous measurements (n = 763) from clinical patients with suspected DIC or coagulation activation. 24.8% (189/763) of samples with elevated FDP were negative for D-dimer assays by the latex agglutination method. Further detailed analysis on randomly-selected discordant samples (n = 41) revealed that the most common reason for the discordance was the lower sensitivity of the semiquantitative latex agglutination method for D-dimer, compared with quantitative enzyme or other latex immunoassay. The other contributing factors to the discordance were accelerated fibrinogenolysis without secondary fibrinolysis, elevated soluble fibrin monomer and rheumatoid factor.
Blood Coagulation Disorders/blood* ; Disseminated Intravascular Coagulation/blood* ; Fibrin Fibrinogen Degradation Products/analysis* ; Human ; Latex Fixation Tests*

Neonatal bleeding is a common problem encountered in nursery rooms or neonatal intensive care units, especially among premature infants. Furthermore, owing to recent remarkable improvement of neonatology, survival rates of preterm neonates have increased; hence, neonatal bleeding cannot be emphasized enough. Since the total blood volume of neonates is small, bleeding can be one of the causes of morbidities and mortalities. Therefore, rapid diagnosis and immediate therapy is urgently needed. The patient's medical history including a familial history of a bleeding disorder or of a previously affected infant who suffered from bleeding along with maternal and neonatal drugs can provide important diagnostic clues. Presence of bleeding with or without petechiae and ecchymoses in a healthy term or late preterm infant with thrombocytopenia but normal prothrombin time and activated partial thromboplastin time strongly suggests a congenital bleeding disorder. For a sick infant who is bleeding from multiple sites, an acquired disorder such as disseminated intravascular coagulation is suspected. Intracranial hemorrhage in term or late preterm infants without a history of birth trauma is highly suggestive of coagulation disorders. The purpose of this review is to summarize recent advances in diagnostic methods is as well as basic concepts of neonatal hemostatic disorders. First, an outline of background information will be presented followed by a discussion of primary and secondary hemostatic disorders as well as inherited and acquired disorders.
Blood Volume ; Disseminated Intravascular Coagulation ; Ecchymosis ; Hemorrhage ; Hemostasis ; Hemostatic Disorders ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care Units, Neonatal ; Intracranial Hemorrhages ; Neonatology ; Nurseries ; Partial Thromboplastin Time ; Parturition ; Prothrombin Time ; Purpura ; Survival Rate ; Thrombocytopenia

Betacoronavirus ; Blood Coagulation Disorders ; epidemiology ; etiology ; Coronavirus Infections ; complications ; Disseminated Intravascular Coagulation ; epidemiology ; etiology ; Fibrin Fibrinogen Degradation Products ; analysis ; Humans ; Pandemics ; Pneumonia, Viral ; complications ; Venous Thromboembolism ; epidemiology ; etiology

Trauma-induced coagulopathy is classified into primary and secondary coagulopathy, with the former elicited by trauma and traumatic shock itself and the latter being acquired coagulopathy induced by anemia, hypothermia, acidosis, and dilution. Primary coagulopathy consists of disseminated intravascular coagulation and acute coagulopathy of trauma shock (ACOTS). The pathophysiology of ACOTS is the suppression of thrombin generation and neutralization of plasminogen activator inhibitor-1 mediated by activated protein C that leads to hypocoagulation and hyperfibrinolysis in the circulation. This review tried to clarify the validity of activated protein C hypothesis that constitutes the main pathophysiology of the ACOTS in experimental trauma models.
Acute Disease ; Animals ; Blood Coagulation Disorders ; etiology ; Disease Models, Animal ; Disseminated Intravascular Coagulation ; etiology ; Humans ; Mice ; Plasminogen Activator Inhibitor 1 ; Protein C ; physiology ; Thrombin ; Wounds and Injuries ; complications

Acute coagulopathy of trauma-shock (ACoTS) occurs in 25% of patients with severe trauma in the early phase, and the mortality of those patients is four-fold higher than patients without coagulopathy. The pathophysiology of this complicated phenomenon has been focused on in recent years. Tissue injury and hypoperfusion, activated protein C and Complements play important roles in the early phase after trauma. While the use of blood products, hypothermia, acidosis and inflammation are the main mechanism in late phase. Supplementing coagulation factors and platelets to improve ACoTS are inefficient. Only positive resuscitation from shock and improving tissue hypoperfusion have expected benefits.
Blood Coagulation Disorders ; etiology ; Complement System Proteins ; physiology ; Disseminated Intravascular Coagulation ; etiology ; Humans ; Hypothermia ; complications ; Inflammation ; complications ; Protein C ; physiology ; Shock, Traumatic ; complications

The Sepsis Coagulopathy Asahi Recombinant LE Thrombomodulin (SCARLET) trial has many defects, and thus cannot be the terminator of recombinant thrombomodulin (rTM). On the contrary, it provides sufficient evidence for further research. Based on analysis focusing on the failure of SCARLET and several previous anticoagulant studies, it is most important for new studies to grasp the following two points: (1) The enrolled cases should have sufficient disease severity and a clear standard for disseminated intravascular coagulation; (2) Heparin should not be used in combination with the investigated drugs. Multiple post-hoc analyses show that no combination of heparin will not increase the risk of thromboembolism. In fact, the combination of heparin can mask the true efficacy of the investigated drug. Due to the complexity of sepsis treatment and the limitations of clinical studies, the results of all treatment studies should be repeatedly verified, rather than be determined at one stroke. Some research conclusions contrary to disease physiology, pharmacology and clinical practice may be deceptive, and should be cautious rather than be simply accepted. On the other hand, the dissenting voices in the "consensus" scene are often well discussed by the authors and should be highly valued.
Humans ; Anticoagulants/therapeutic use* ; Thrombomodulin/therapeutic use* ; Blood Coagulation Disorders ; Disseminated Intravascular Coagulation/drug therapy* ; Sepsis/drug therapy* ; Heparin/therapeutic use* ; Recombinant Proteins

As the most abundant component of coagulation system, fibrinogen not only takes part in clotting, but also works as one of acute phase proteins, which participates in many physiological and pathophysiological processes. Studies of fibrinogen abnormalities contribute to understand the molecular basis of disorders of fibrinogen protein function and metabolism, caused mainly by gene mutation, commonly associated with bleeding, thrombophilia, or both. Diseases affecting fibrinogen could be classified to the acquired or inherited disease. In this review, the research progress on the molecular basis, possible action mechanism of the hereditary fibrinogen abnormalities and its clinical research are summarized.
Afibrinogenemia ; genetics ; Blood Coagulation Disorders ; genetics ; Humans ; Mutation

<b>OBJECTIVEb>To analyze the gene mutation in two pedigrees of inherited coagulation factor VII (FVII) deficiency, and investigate the relationship between the genotype and phenotype.

<b>METHODb>The coagulation function and coagulation factors activity of probands were detected for phenotype diagnosis, all exons and junctions of FVII gene from the family members' genomic DNA were amplified using polymerase chain reaction (PCR), and detected the gene mutation by direct sequencing. Mutations were confirmed by reverse sequencing.

<b>RESULTb>The prothrombin time (PT) of proband 1 was 265.2 s, FVII:C was 22% and the PT of proband 2 was > 120 s, FVII:C was 1%. Homozygous 17844G→A mutation in No. 8 exon of FVII gene was identified in the proband 1 resulting in Gly343Ser, and heterozygosity for the same mutations were confirmed in his parents and a sister. The proband 2 was compound heterozygous, one mutation was the same as the proband 1 but was a heterozygosity that can also found in his mother and brother; the other heterozygosity mutation was located on No. 8 exon 18055G→A that resulted in Gln413Arg which was inherited from his father.

<b>CONCLUSIONb>No. 8 exon of FVII gene encodes catalytic domain. Mutation found in those domain could change the FVII catalytic domain spatial structure, affected FVII function and stability, and the sufferer of homozygote and compound heterozygous may have clinical bleeding tendency. Almost no clinical findings in simple heterozygotes, however, a few of heterozygotes could have a tendency of bleeding because of genetic polymorphism which would reduce the FVII:C.

Blood Coagulation Disorders ; blood ; genetics ; Child, Preschool ; DNA Mutational Analysis ; Factor VII ; genetics ; Factor VII Deficiency ; blood ; genetics ; Heterozygote ; Homozygote ; Humans ; Infant ; Male ; Molecular Sequence Data ; Mutation ; Pedigree ; Polymerase Chain Reaction ; Prothrombin Time

Disseminated intravascular coagulation (DIC) is a pathological syndrome in which activation of coagulation cascade leads to fibrin clot formation, consumption of platelets and coagulation factors, and secondary fibrinolysis. We report a case of severe postoperative hemorrhagic diathesis due to DIC. A 59-year-old man was scheduled for reduction of tibia fracture and anatrophic nephrolithotomy of staghorn calculi. On the fifth postoperative day, second operation was performed for nephrectomy due to perirenal hematoma. Two days later, third operation was performed for hemostasis because of the continuous bleeding. Coagulation tests showed positive DIC profiles of thrombocytopenia, hypofibrinogenemia, increased fibrin degradation products, and prolonged prothrombin time and thrombin time. The patient recovered uneventfully and discharged on the 59th postoperative day.
Blood Coagulation Factors ; Calculi ; Dacarbazine ; Disseminated Intravascular Coagulation* ; Fibrin ; Fibrin Fibrinogen Degradation Products ; Fibrinolysis ; Hematoma ; Hemorrhage* ; Hemorrhagic Disorders ; Hemostasis ; Humans ; Middle Aged ; Nephrectomy ; Postoperative Complications ; Prothrombin Time ; Thrombin Time ; Thrombocytopenia ; Tibia