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Clinical Rheumatology May 2024The objective of this study was to analyze the changes in plasma fibrinogen (FIB) levels during tocilizumab (TCZ) treatment in patients with rheumatic diseases, to...
OBJECTIVE
The objective of this study was to analyze the changes in plasma fibrinogen (FIB) levels during tocilizumab (TCZ) treatment in patients with rheumatic diseases, to clarify the incidence of hypofibrinogenemia and its possible risk factors, and to establish a nomogram model for predicting the probability of hypofibrinogenemia in rheumatoid arthritis (RA) patients treated with TCZ.
METHODS
Clinical data of patients treated with TCZ at the Department of Rheumatology and Immunology, the First Affiliated Hospital of Xi'an Jiaotong University from January 2014 to October 2021 were retrospectively analyzed to observe the incidence of hypofibrinogenemia in several rheumatic diseases at different time points. The risk factor of hypofibrinogenemia in RA patients treated with TCZ was determined by using Cox regression analysis. Based on the results of Cox regression analysis, a nomogram for predicting the probability of hypofibrinogenemia in rheumatoid arthritis (RA) patients treated with TCZ was established and validated through RStudio software.
RESULTS
A total of 83 TCZ-treated patients were enrolled in this study, and 32 (38.55%) patients developed hypofibrinogenemia during TCZ treatment. There were 8 males and 24 females in the FIB-reduced group, with an average age of 44.88 ± 18.39 years. Hypofibrinogenemia was most common in TCZ-treated patients with takayasu arteritis (TA) and RA. Hypofibrinogenemia typically occured within 3 months after TCZ treatment. In RA patients treated with TCZ, platelet distribution width, parathyroid hormone, bone mineral density, tender joint count, and swollen joint count were independent risk factors for the occurrence of hypofibrinogenemia. The nomogram based on the above risk factors could effectively predict the probability of hypofibrinogenemia in RA patients receiving TCZ.
CONCLUSION
Although bleeding symptoms were not observed in this study, the incidence of hypofibrinogenemia remained high after TCZ treatment, usually occurring within 3 months of treatment. Therefore, it is necessary to monitor FIB levels during TCZ treatment. In addition, clinicians can use the nomogram model developed from this study to predict the incidence of hypofibrinogenemia after TCZ treatment in RA patients. Key Points • Hypofibrinogenemia often occurs during TCZ treatment for rheumatic diseases. • PDW, PTH, BMD, tender joint count, and swollen joint count are risk factors for the occurrence of hypofibrinogenemia. • It is necessary to monitor FIB levels during TCZ treatment to avoid bleeding tendency.
Topics: Male; Female; Humans; Adult; Middle Aged; Antirheumatic Agents; Afibrinogenemia; Retrospective Studies; Arthritis, Rheumatoid; Risk Factors; Treatment Outcome; Antibodies, Monoclonal, Humanized
PubMed: 38494557
DOI: 10.1007/s10067-024-06937-0 -
Thrombosis Research Apr 2024Tigecycline-associated hypofibrinogenemia has been reported as an important adverse effect in recent years, but controlled studies minimizing confounding factors are...
BACKGROUND
Tigecycline-associated hypofibrinogenemia has been reported as an important adverse effect in recent years, but controlled studies minimizing confounding factors are needed. The objective of our study was to assess changes in fibrinogen levels in patients for hospitalization, comparing two antibiotic episodes (tigecycline and other) within the same patients.
METHODS
The retrospective, self-controlled case series study was conducted at our University Hospitals. The study compared the change in fibrinogen levels during the patient's hospitalization for tigecycline (TigePer) and another antibiotic period (OtherPer). In addition, bleeding events, bleeding risk (determined by the IMPROVE bleeding risk score), as well as 15- and 30-day mortality rates between TigePer and OtherPer were compared.
RESULTS
The study enrolled 50 patients with 100 episodes of antibiotic treatment. The median age (interquartile range) of the patients was 68.5 (21.5) years, and 38 % were female. As compared to OtherPer, TigePer had a statistically significant reduction in fibrinogen levels (p < 0.001), with a hypofibrinogenemia rate of 40 % in TigePer as compared to 2 % in OtherPer (p < 0.001). TigePer demonstrated a significantly higher 15-day mortality rate (p = 0.006). No significant differences were observed between the two periods in terms of bleeding risk, rate of bleeding events, and 30-day mortality rate (p > 0.05).
CONCLUSION
Hypofibrinogenemia and other coagulopathies, without associated bleeding events, are more frequently observed in patients receiving tigecycline. Therefore, it is crucial for clinicians to monitor fibrinogen levels during tigecycline use.
Topics: Humans; Female; Aged; Male; Afibrinogenemia; Tigecycline; Fibrinogen; Retrospective Studies; Anti-Bacterial Agents; Hemorrhage
PubMed: 38452447
DOI: 10.1016/j.thromres.2024.03.003 -
Brazilian Journal of Cardiovascular... Feb 2024Inflammatory and immunological factors play pivotal roles in the prognosis of acute type A aortic dissection. We aimed to evaluate the prognostic values of...
INTRODUCTION
Inflammatory and immunological factors play pivotal roles in the prognosis of acute type A aortic dissection. We aimed to evaluate the prognostic values of immune-inflammatory parameters in acute type A aortic dissection patients after surgery.
METHODS
A total of 127 acute type A aortic dissection patients were included. Perioperative clinical data were collected through the hospital's information system. The outcomes studied were delayed extubation, reintubation, and 30-day mortality. Multivariate logistic regression analysis and receiver operating characteristic analysis were used to screen the risk factors of poor prognosis.
RESULTS
Of all participants, 94 were male, and mean age was 51.95±11.89 years. The postoperative prognostic nutritional indexes were lower in delayed extubation patients, reintubation patients, and patients who died within 30 days. After multivariate regression analysis, the postoperative prognostic nutritional index was a protective parameter of poor prognosis. The odds ratios (95% confidence interval) of postoperative prognostic nutritional index were 0.898 (0.815, 0.989) for delayed extubation and 0.792 (0.696, 0.901) for 30-day mortality. Low postoperative fibrinogen could also well predict poor clinical outcomes. The odds ratios (95% confidence interval) of postoperative fibrinogen were 0.487 (0.291, 0.813) for delayed extubation, 0.292 (0.124, 0.687) for reintubation, and 0.249 (0.093, 0.669) for 30-day mortality.
CONCLUSION
Postoperative prognostic nutritional index and postoperative fibrinogen could be two promising markers to identify poor prognosis of acute type A aortic dissection patients after surgery.
Topics: Humans; Male; Adult; Middle Aged; Female; Fibrinogen; Prognosis; Nutrition Assessment; Retrospective Studies; Aortic Dissection; Risk Factors
PubMed: 38426429
DOI: 10.21470/1678-9741-2022-0185 -
Neurosurgical Review Feb 2024In patients affected by traumatic brain injury (TBI), hypofibrinogenemia within the initial hours of trauma can be expected due to vascular and inflammatory changes. In...
In patients affected by traumatic brain injury (TBI), hypofibrinogenemia within the initial hours of trauma can be expected due to vascular and inflammatory changes. In this study, we aimed to evaluate the effect of hypofibrinogenemia on the in-hospital mortality and 6-month functional outcomes of TBI patients, admitted to Rajaee Hospital, a referral trauma center in Shiraz, Iran. This study included all TBI patients admitted to our center who had no prior history of coagulopathy or any systemic disease, were alive on arrival, and had not received any blood product before admission. On admission, hospitalization, imaging, and 6-month follow-up information of included patients were extracted from the TBI registry database. The baseline characteristics of patients with fibrinogen levels of less than 150 mg/dL were compared with the cases with higher levels. To assess the effect of low fibrinogen levels on in-hospital mortality, a uni- and multivariate was conducted between those who died in hospital and survivors. Based on the 6-month GOSE score of patients, those with GOSE < 4 (unfavorable outcome) were compared with those with a favorable outcome. A total of 3049 patients (84.3% male, 15.7% female), with a mean age of 39.25 ± 18.87, met the eligibility criteria of this study. 494 patients had fibrinogen levels < 150 mg/dl, who were mostly younger and had lower average GCS scores in comparison to cases with higher fibrinogen levels. By comparison of the patients who died during hospitalization and survivors, it was shown that fibrinogen < 150 mg/dl is among the prognostic factors for in-hospital mortality (OR:1.75, CI: 1.32:2.34, P-value < 0.001), while the comparison between patients with the favorable and unfavorable functional outcome at 6-month follow-up, was not in favor of prognostic effect of low fibrinogen level (OR: 0.80, CI: 0.58: 1.11, P-value: 0.19). Hypofibrinogenemia is associated with in-hospital mortality of TBI patients, along with known factors such as higher age and lower initial GCS score. However, it is not among the prognostic factors of midterm functional outcome.
Topics: Humans; Male; Female; Young Adult; Adult; Middle Aged; Afibrinogenemia; Hospital Mortality; Glasgow Coma Scale; Brain Injuries, Traumatic; Prognosis; Fibrinogen
PubMed: 38413402
DOI: 10.1007/s10143-024-02326-y -
Wilderness & Environmental Medicine Mar 2024is a Crotaline viperid species of Central America and Northern South America. The characteristics of its envenomation have not been well established. We present...
INTRODUCTION
is a Crotaline viperid species of Central America and Northern South America. The characteristics of its envenomation have not been well established. We present clinical characteristics of human cases evaluated and treated in a hospital in southwestern Colombia.
METHODS
We evaluated data from patients who suffered envenomation and were seen at Fundación Valle del Lili Hospital, Cali, Colombia between 2011 and 2022.
RESULTS
Eight patients were included, with a median age of 24 years. Snakebites occurred in rural areas. Six (75%) patients were bitten on the upper extremities in relation to the arboreal habits of this animal. The most common symptoms were pain and edema (= 8, 100%), ecchymoses ( = 2, 25%), and paresthesia ( = 2, 25%). The most common systemic findings were hypofibrinogenemia (= 8, 100%) and prolonged prothrombin time in five patients ( = 5, 62.5%). All were treated with polyvalent antivenom for Colombian snakes, with a good response and outcome.
CONCLUSIONS
Most bite sites from were on the upper limbs. All patients had both local manifestations, including edema, pain, and systemic effects with hypofibrinogenemia, but none had systemic bleeding. Every patient received antivenom and had favorable outcomes.
Topics: Animals; Humans; Young Adult; Adult; Colombia; Antivenins; Afibrinogenemia; Crotalinae; Bothrops; Pain; Edema
PubMed: 38379478
DOI: 10.1177/10806032231222353 -
Hereditas Feb 2024Congenital fibrinogen disorders are a group of coagulation deficiencies caused by fibrinogen defects and are divided into four types, including afibrinogenemia,...
Congenital fibrinogen disorders are a group of coagulation deficiencies caused by fibrinogen defects and are divided into four types, including afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia. In this study, we collected a family with hypofibrinogenemia, and genetics analysis identify a novel pathogenic variants (c.668G > C, p.Arg223Thr) in the FGG gene. And electron microscope observation revealed significant changes in the ultrastructure of fibrin of the proband. Our research expands the phenotypic and genetic spectrum associated with the FGG gene, which would facilitate in genetic counselling and prenatal genetic diagnosis.
Topics: Humans; Afibrinogenemia; Asian People; China; Fibrinogen; Mutation
PubMed: 38374144
DOI: 10.1186/s41065-024-00313-3 -
Blood Coagulation & Fibrinolysis : An... Apr 2024An elderly woman with light chain myeloma presented with prolonged epistaxis and extensive cutaneous haematomas: her kappa/lambda ratio was high at 395, her coagulation...
An elderly woman with light chain myeloma presented with prolonged epistaxis and extensive cutaneous haematomas: her kappa/lambda ratio was high at 395, her coagulation screen, thrombin and reptilase times were abnormal, her FV and FX were in the low range in the absence of specific inhibitors, her Clauss fibrinogen was low at 0.95 g/l but antigenic FNG was 1.58 g/l. The patient denied treatment and died of progressive renal failure. We wish to describe the unusual association of FX and FV deficiency co-existing with an acquired dysfibrinogenaemia.
Topics: Aged; Female; Humans; Afibrinogenemia; Factor V; Factor X Deficiency; Fibrinogen; Multiple Myeloma
PubMed: 38358896
DOI: 10.1097/MBC.0000000000001280 -
Blood Advances Mar 2024Congenital fibrinogen deficiency (CFD) is a rare bleeding disorder caused by mutations in FGA, FGB, and FGG. We sought to comprehensively characterize patients with CFD...
Congenital fibrinogen deficiency (CFD) is a rare bleeding disorder caused by mutations in FGA, FGB, and FGG. We sought to comprehensively characterize patients with CFD using PRO-RBDD (Prospective Rare Bleeding Disorders Database). Clinical phenotypes, laboratory, and genetic features were investigated using retrospective data from the PRO-RBDD. Patients were classified from asymptomatic to grade 3 based on their bleeding severity. In addition, FGA, FGB, and FGG were sequenced to find causative variants. A total of 166 CFD cases from 16 countries were included, of whom 123 (30 afibrinogenemia, 33 hypofibrinogenemia, 55 dysfibrinogenemia, and 5 hypodysfibrinogenemia) were well characterized. Considering the previously established factor activity and antigen level thresholds, bleeding severity was correctly identified in 58% of the cases. The rates of thrombotic events among afibrinogenemic and hypofibrinogenemic patients were relatively similar (11% and 10%, respectively) and surprisingly higher than in dysfibrinogenemic cases. The rate of spontaneous abortions among 68 pregnancies was 31%, including 86% in dysfibrinogenemic women and 14% with hypofibrinogenemia. Eighty-six patients received treatment (69 on-demand and/or 17 on prophylaxis), with fibrinogen concentrates being the most frequently used product. Genetic analysis was available for 91 cases and 41 distinct variants were identified. Hotspot variants (FGG, p.Arg301Cys/His and FGA, p.Arg35Cys/His) were present in 51% of dysfibrinogenemia. Obstetric complications were commonly observed in dysfibrinogenemia. This large multicenter study provided a comprehensive insight into the clinical, laboratory, and genetic history of patients with CFDs. We conclude that bleeding severity grades were in agreement with the established factor activity threshold in nearly half of the cases with quantitative defects.
Topics: Humans; Female; Fibrinogen; Afibrinogenemia; Prospective Studies; Retrospective Studies; Hemorrhage; Hemostatics
PubMed: 38286442
DOI: 10.1182/bloodadvances.2023012186 -
Journal of Thrombosis and Haemostasis :... May 2024Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and/or qualitative fibrinogen deficiencies. The spectrum of molecular... (Review)
Review
Congenital fibrinogen disorders (CFDs) are a heterogeneous group of rare congenital quantitative and/or qualitative fibrinogen deficiencies. The spectrum of molecular anomalies is broad, leading to several subtypes of fibrinogen disorders (ie, afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia). Pregnancy in women with CFDs is a high-risk clinical situation, with an increased tendency for miscarriages, bleeding, and thrombosis. Even though it is well established that management of such pregnancies requires a multidisciplinary approach involving specialists (hematologists and maternal/fetal medicine experts with expertise in the management of inherited bleeding disorders), specific guidelines are lacking. In this International Society on Thrombosis and Haemostasis (ISTH) Scientific and Standardization Committee communication, we aim to propose an expert consensus opinion with literature evidence where available on the strategy for management of pregnancy, delivery, and puerperium in CFDs.
Topics: Humans; Pregnancy; Female; Afibrinogenemia; Pregnancy Complications, Hematologic; Fibrinogen; Factor XIII; Delivery, Obstetric; Consensus
PubMed: 38266678
DOI: 10.1016/j.jtha.2024.01.008 -
Blood Coagulation & Fibrinolysis : An... Mar 2024Rotational thromboelastometry (ROTEM) is a global hemostasis assay. The diagnosis added value of ROTEM in congenital dysfibrinogenemia remains to be established. The aim...
Rotational thromboelastometry (ROTEM) is a global hemostasis assay. The diagnosis added value of ROTEM in congenital dysfibrinogenemia remains to be established. The aim of this study was to analyze clot formation by ROTEM in a cohort of dysfibrinogenemic patients and to establish correlations with genotype, clinical features, and coagulation parameters. The study included genetically confirmed congenital dysfibrinogenemia cases (n = 63) and healthy controls ( n = 50). EXTEM, INTEM, FIBTEM tests were used to measure ROTEM parameters, that is, clotting time (CT), clot formation time (CFT), maximal clot firmness (MCF) and amplitude 10 min after CT (A10). The ISTH bleeding assessment tool was used to determine bleeding episodes. CT (INTEM) was statistically significantly shorter in congenital dysfibrinogenemia patients compared to controls while CFT (EXTEM) was prolonged. Patients's MCF in EXTEM, INTEM, and FIBTEM were similar to controls while A10 (FIBTEM) was statistically significantly lower. Fibrinogen activity was positively correlated with fibrinogen antigen, A10 and MCF in all three assays. Bleeding phenotypes were observed in 23 (36.5%) patients. Only CFT in EXTEM and CT in INTEM were statistically different in patients with bleeding phenotype versus controls. Carriers of the FGA mutation p.Arg35His had a CT (EXTEM) slightly prolonged and a reduced A10 (FIBTEM) compared to controls. Some ROTEM parameters were able to distinguish congenital dysfibrinogenemia patients from controls, and patients with a bleeding phenotype. Prolonged CFT in EXTEM were associated with congenital dysfibrinogenemia and bleeding phenotype. Bleeding episodes in most patients were generally mild and prevalence of thrombosis was very low.
Topics: Humans; Thrombelastography; Prospective Studies; Blood Coagulation Tests; Hemorrhage; Fibrinogen; Afibrinogenemia; Piperidones; Benzeneacetamides
PubMed: 38251440
DOI: 10.1097/MBC.0000000000001274