Abnormal coagulation after hepatectomy in patients with normal preoperative coagulation function.

BACKGROUND: To explore the risk factors for postoperative abnormal coagulation (PAC) and establish a predictive model for patients with normal preoperative coagulation function who underwent hepatectomy. MATERIALS AND METHODS: A total of 661 patients with normal preoperative coagulation function who underwent hepatectomy between January 2015 and December 2021 at the First Affiliated Hospital of Sun Yat-sen University were divided into two groups: the postoperative abnormal coagulation group (PAC group, n = 362) and the normal coagulation group (non-PAC group, n = 299). Univariate and multivariate logistic analyses were used to identify the risk factors for PAC. RESULTS: The incidence of PAC in 661 patients who underwent hepatectomy was 54.8% (362/661). The least absolute shrinkage and selection operator (LASSO) method was used for multivariate logistic regression analysis. The preoperative international normalized ratio (INR), intraoperative succinyl gelatin infusion and major hepatectomy were found to be independent risk factors for PAC. A nomogram for predicting the PAC after hepatectomy was constructed. The model presented a receiver operating characteristic (ROC) curve of 0.742 (95% confidence interval (CI): 0.697-0.786) in the training cohort. The validation set demonstrated a promising ROC of 0.711 (95% CI: 0.639-0.783), and the calibration curve closely approximated the true incidence. Decision curve analysis (DCA) was performed to assess the clinical usefulness of the predictive model. The risk of PAC increased when the preoperative international normalized ratio (INR) was greater than 1.025 and the volume of intraoperative succinyl gelatin infusion was greater than 1500 ml. CONCLUSION: The PAC is closely related to the preoperative INR, intraoperative succinyl gelatin infusion and major hepatectomy. A three-factor prediction model was successfully established for predicting the PAC after hepatectomy.

Rare bleeding disorders: Real-world data from a Spanish tertiary hospital.

INTRODUCTION: Due to their low prevalence, rare bleeding disorders (RBDs) remain poorly characterized. AIM: To gain insight of RBDs through our clinical practice. METHODS: Retrospective study of the medical records of RBD patients followed up at the Central University Hospital of Asturias between January 2019 and December 2022. RESULTS: A total of 149 patients were included. Factor (F) VII (44 %) and FXI (40 %) deficiencies were the most common diagnosed coagulopathies. Most of the patients were asymptomatic (60.4 %) and the most frequent type of bleeding were mucocutaneous and after surgery. All replacement treatments were administered on demand and no patient was on a prophylaxis regimen. Currently available products were safe; allergic reactions after administration of plasma were the most frequent complication. Genetic analysis, carried out on 55 patients (37 %), showed that the most frequent mutations in RBDs are of missense type (71.9 %). We identified 11 different novel genetic alterations in affected genes. The c.802C > T (p.Arg268Cys) variant, previously described, was identified in 71 % (15 of 21) of the patients with FXI deficiency genotyped and none were related (probable founder effect). CONCLUSION: Our study on an unusual large single center cohort of RBD patients portrays location-dependent distinct genetic drives and clinical practice particularities.

The prevalence and risk factors of coagulopathy in pediatric patients undergoing surgery for epilepsy.

OBJECTIVE: Hematological consequences of novel antiseizure medications (ASMs) or combined therapies are rarely reported, especially in pediatric patients undergoing surgery for epilepsy. This study aimed to assess the prevalence and risk factors of coagulation dysfunction in this population and evaluate their relationship with intra- and postoperative bleeding. METHODS: Three hundred ninety children who underwent surgery for epilepsy and 104 children without epilepsy who underwent nonepilepsy surgery at the authors' center were included in the study. The authors retrospectively collected and analyzed the following clinical data: sex, age, weight, course of epilepsy, antiseizure therapy, first laboratory data after admission, and transfusion-related data. RESULTS: ASMs were responsible for the higher incidence of coagulation dysfunction in pediatric epilepsy surgery patients. Low body weight (OR 0.95, 95% CI 0.92-0.98) and valproic acid (VPA) therapy (OR 5.13, 95% CI 3.25-8.22) were the most relevant factors leading to coagulation dysfunction. The most common hematological side effects of VPA were thrombocytopenia and hypofibrinogenemia, whereas low body weight was only associated with hypofibrinogenemia. Both VPA and low body weight increased the need for intra- or postoperative transfusion (p < 0.001). CONCLUSIONS: Pediatric epilepsy surgery patients often take multiple ASMs, resulting in an increased incidence of coagulopathy. VPA levels and low body weight were found to be the main influential factors associated with an increased risk of coagulation dysfunction. Platelet and fibrinogen levels were the main indices that were affected. Both VPA and low body weight were relevant to additional surgery-related transfusion, necessitating the need for increased awareness of preoperative coagulopathy before pediatric epilepsy surgery. Clinical trial registration no.: NCT05675254 (ClinicalTrials.gov).

Postoperative coagulopathy among otherwise healthy pediatric patients undergoing open craniosynostosis repair: a retrospective study.

Significant blood loss and resultant transfusion may lead to coagulopathy. The need for routine transfusion of non-RBC blood products in healthy pediatric patients suffering significant, yet controlled, intra-operative blood loss is controversial. Open craniosynostosis surgery is often associated with significant intra-operative blood loss and transfusion, and routinely preformed on otherwise healthy pediatric patients. Therefore, we found it as a useful model for our study, which aimed to assess the need for routine transfusion of non-RBC blood products in healthy pediatric patients suffering significant intra-operative blood loss. We conducted a retrospective cohort study of otherwise healthy pediatric patients, undergoing open craniosynostosis surgery and transfused solely with packed red blood cells (pRBCs) in a single large-volume tertiary surgical center, between January 2010 and December 2021. Among 457 eligible patients, 34 (7.4%) developed significant postoperative coagulopathy. Median [IQR] intra-operative pRBC transfusion volume was 17.4 ml kg-1 [13.3, 23.1]. Patients who developed coagulopathy did not have higher postoperative pRBC transfusion rate (8.8% vs 3.8%, P = 0.16) or volume (median [IQR], 0 [0, 0] vs 0 [0, 0] ml, P = 0.15), nor higher hospital LOS (5 [4, 5] vs 5 [4, 5] days, P = 0.66). ICU LOS was 0.8 [0.7, 1] vs 0.7 [0.6, 0.8] days (P = 0.02), a difference of no clinical significance.  Conclusions: The incidence of significant coagulopathy after craniosynostosis surgery was low, and not associated with clinically important complications. In otherwise healthy pediatric patients, even significant intra-operative blood loss can be safely managed solely with intravenous fluids and pRBC transfusion. What is Known: • Significant intra-operative blood loss and resultant transfusion may lead to postoperative coagulopathy. • There are potential deleterious effects from both coagulopathy and administration of blood products. What is New: • Open craniosynostosis corrective surgery is a useful model for studying coagulopathy after significant intra-operative blood loss and transfusion in otherwise healthy children. • Under certain conditions, in otherwise healthy pediatric patients, even significant intra-operative blood loss can be safely treated with intravenous fluids and pRBC transfusion alone, with no clinically significant postoperative coagulopathy or its complications.

Analysis of Risk Factors of Coagulation Dysfunction and Hemorrhage in Newly Diagnosed Hyperleukocytic Acute Myeloma Leukemia.

Purpose: To explore whether and why abnormal coagulation function and hemorrhage can appear in patients with hyperleukocytic acute myeloid leukemia (HAML). Method: We retrospectively reviewed the charts of 724 acute myeloid leukemia (AML) patients admitted with a white blood cell count of >100 × 109/L between 2010 and 2019 in order to analyze the coagulation index of patients with HAML and explore the correlation of abnormal coagulation. Result: Prothrombin time (PT) was extended in group HAML compared with group non-HAML. Respiratory failure, intracranial hemorrhage, and infection were more common in the HAML group. Among the 76 HAML patients, there were 33 patients who had ≥3 abnormal items of coagulation index, and 51.5% of them had level 2 hemorrhage which was more than level 0 hemorrhage, and the difference is statistically significant (P < 0.01). Similarly, we can discover that 40.9% of patients with 2 abnormal items had level 2 hemorrhage in contrast to 0 abnormal items. The use of hydroxyurea had a significant effect on PT and D-dimer (DD). Survival analysis by the Kaplan-Meier method showed that there were statistically significant differences in white blood cell (WBC) count＞200 × 109//L and DD. Abnormal PT is associated with WBC count＞200 × 109//L, and abnormal activated partial thromboplastin time (APTT) is associated with HLA-DR mutation. Infection and respiratory failure were independent influencing factors for the coagulation of patients. Conclusion: DD had a marked effect on the survival rate. Infection and respiratory failure were independent influencing factors for the coagulation of patients.

Bleeding disorders in implant dentistry: a narrative review and a treatment guide.

PURPOSE: Considering a high prevalence of congenital and especially acquired bleeding disorders, their heterogeneity and the multitude of possible treatments strategies, a review of the scientific data on this topic is needed to implement a treatment guide for healthcare professionals. METHODS: A selective literature review was performed via PubMed for articles describing oral surgery / dental implant procedures in patients with congenital and acquired bleeding disorders. Out of the existing literature, potential treatment algorithms were extrapolated. RESULTS: In order to assess the susceptibility to bleeding, risk stratification can be used for both congenital and acquired coagulation disorders. This risk stratification, together with an appropriate therapeutic pathway, allows for an adequate and individualized therapy for each patient. A central point is the close interdisciplinary cooperation with specialists. In addition to the discontinuation or replacement of existing treatment modalities, local hemostyptic measures are of primary importance. If local measures are not sufficient, systemically administered substances such as desmopressin and blood products have to be used. CONCLUSIONS: Despite the limited evidence, a treatment guide could be developed by means of this narrative review to improve safety for patients and practitioners. Prospective randomized controlled trials are needed to allow the implementation of official evidence-based guidelines.

Safety of Laparoscopic Appendectomy in the Setting of Coagulopathy: An ACS-NSQIP Study.

INTRODUCTION: Patients with coagulopathy requiring emergent appendectomy constitute a challenging patient population. It is unclear whether laparoscopic appendectomy (LA) is as safe as open appendectomy (OA) in these patients. METHODS: We queried the ACS-NSQIP database for adults with coagulopathy undergoing emergent appendectomy from 2014 to 2017. Demographic characteristics and operative outcomes were compared between the two groups. Propensity weighting for LA versus OA was estimated using augmented inverse probability of treatment weights (AIPW). RESULTS: A total of 137,429 patients were included, of which 7049 (5%) had coagulopathy. In patients with coagulopathy, LA was the most common approach (89%). After AIPW, there was no difference in the adjusted risk of either postoperative transfusion or 30-day reoperation between OA and LA. LA was associated with reduced operative time (56 versus 75 min), length of stay (3.5 versus 7.0 d), and surgical site infection rate (6% versus 13%) compared to OA. CONCLUSIONS: Patients with coagulopathy represent a significant proportion of those undergoing an appendectomy. The majority of patients with coagulopathy who require appendectomy undergo LA, and this approach appears to be safe with regard to transfusion requirement and reoperation.

Assessment of need for hemostatic evaluation in patients taking valproic acid: A retrospective cross-sectional study.

INTRODUCTION: Valproic acid (VPA) is a frequently prescribed anti-epileptic drug. Since its introduction side effects on hemostasis are reported. However, studies show conflicting results, and the clinical relevance is questioned. We aimed to determine the coagulopathies induced by VPA in patients who undergo high-risk surgery. The study results warrant attention to this issue, which might contribute to reducing bleeding complications in future patients. METHODS: Between January 2012 and August 2020, 73 consecutive patients using VPA were retrospectively included. Extensive laboratory hemostatic assessment (including platelet function tests) was performed before elective high-risk surgery. Patient characteristics, details of VPA treatment, and laboratory results were extracted from medical records. RESULTS: 46.6% of the patients using VPA (n = 73) showed coagulopathy. Mainly, platelet function disorder was found (36.4%). Thrombocytopenia was seen in 9.6% of the patients. Data suggested that the incidence of coagulopathies was almost twice as high in children as compared to adults and hypofibrinogenemia was only demonstrated in children. No association was found between the incidence of coagulopathies and VPA dosage (mg/kg/day). CONCLUSION: A considerable number of patients using VPA were diagnosed with coagulopathy, especially platelet function disorder. Further prospective studies are needed to confirm the need for comprehensive laboratory testing before elective high-risk surgery in these patients.

Sex dimorphisms in coagulation characteristics in the pediatric trauma population appear after puberty.

BACKGROUND: The role of age and sex in mediating coagulation characteristics in injured children is not well defined. We hypothesize that thromboelastography (TEG) profiles are equivalent across sex in younger children and diverge after puberty. METHODS: Consecutive trauma patients younger than 18 years were identified from a university-affiliated, Level I, pediatric trauma center (2016-2020) database. Demographics, injury characteristics, and TEG parameters were recorded. Children were categorized by sex and age (younger, ≤10 years; older, ≥11 years). Baseline characteristics, outcomes, and TEG parameters were compared using nonparametric tests as appropriate. To account for the effects of confounding variables, analysis of covariance was performed controlling for Injury Severity Score (ISS), admission Glasgow Coma Scale score, and pediatric age-adjusted shock index. RESULTS: Six hundred forty-seven subjects were identified (70.2% male, median ISS, 10; interquartile range, 5-24; blunt mechanism, 75.4%). Among 395 younger children (≤10 years), there were no differences in TEG characteristics between sexes. Among 252 adolescents (≥11 years), males had greater kinetic times (1.8 vs. 1.4 min; p < 0.001), decreased alpha angles (69.6° vs73.7°; p < 0.001), and lower maximum amplitudes (59.4 vs. 61.5 mm; p = 0.01). Fibrinolysis was significantly lower in older females compared with younger females (0.4% vs. 1.5%, p < 0.001) and age-matched males (0.4% vs. 1.0%, p = 0.02). Compared with younger male children, adolescent males had greater kinetic times (1.8 vs. 1.4 min; p < 0.001), decreased alpha angles (73.5° vs. 69.6°, p < 0.001), lower maximum amplitudes (59.4 vs. 62 mm, p < 0.001), and less fibrinolysis (1.0% vs. 1.3%, p = 0.03). This interaction persisted after controlling for ISS, Glasgow Coma Scale, and pediatric age-adjusted shock index. CONCLUSION: Sex dimorphisms in TEG coagulation profiles appear after puberty. This divergence appears to be driven by a shift in male coagulation profiles to a relatively hypocoagulable state and female coagulation profiles to a relatively hypercoagulable state after puberty. LEVEL OF EVIDENCE: Prognostic and Epidemiologic, Level III.

Detecting coagulopathy in pediatric patients with post-tonsillectomy hemorrhage.

INTRODUCTION: Post-tonsillectomy hemorrhage (PTH) is a serious complication after a tonsillectomy. Utility of lab work at presentation for PTH was low. This study aims to determine the frequency and type of labs drawn at emergency department (ED) presentation and assess the incidence of uncovering a previously unidentified coagulopathy. METHODS: A retrospective chart review was performed on pediatric patients who were seen in the ED after tonsillectomy at a tertiary care children's hospital from 2017 to 2019. Exclusion criteria were the following: no tonsillar bleed, history of known coagulopathy, treated by outside provider, ≥18 years old. Lab work included complete blood count (CBC), prothrombin time (PT), partial thromboplastin time (PTT), and von Willebrand factor (VWF). RESULTS: 364/723 (50.3%) patients met inclusion criteria. 179/364 (49.2%) patients were male and 309/364 (84.9%) patients were Caucasian. Average age at surgery was 8.12 years (SD = 4.0) and average post-operative day at presentation was 6 days (SD = 2.1). Operative control of bleed was performed in 68/364 (18.7%) patients. 334/364 (91.8%) patients had labs drawn in the ED. 64/334 (19.1%) patients were anemic (hemoglobin (Hgb) < 11), 46/334 (13.8%) patients had thrombocytosis (platelets>450,000), 10/334 (3.0%) had elevated PTT and 8/334 (2.4%) had elevated PT. Hematology was consulted in 14/364 (3.8%) patients of whom 6/14 were diagnosed with von Willebrand disease and 1/14 with factor VII deficiency. Aminocaproic acid was used in 8/364 (2.2%) patients due to elevated PTT in 3/8 and multiple episodes of bleeding in 5/8.3/364 (0.8%) patients needed a blood transfusion. No difference was found in incidence of abnormal lab work in patients who did and did not need operative control of bleed (p = .125). Of the 334 patients who had ED labs drawn, 7 (2.1%) had an uncovered coagulopathy. CONCLUSION: Uncovering incidental coagulopathies is rare in patients who present with PTH. Though anemia was the most common abnormality noted, only a small percentage required transfusion, with all having abnormal vital signs. Thrombocytosis was the next common abnormality, and this can be seen in an inflammatory state. Developing algorithms is necessary to better guide appropriate lab work in patients who present with PTH and to provide optimal value of care to patients.

[Breast cancer-related thrombotic microangiopathy: A review]. / Cancer du sein et microangiopathies thrombotiques paranéoplasiques.

Thrombotic Microangiopathies (TM) have been described since the 1960s. They are characterized by presence of mechanical haemolytic anemia associated with peripheral thrombocytopenia. TM in cancer can be related to several causes, whose cancer himself: cancer-related microangiopathic haemolytic anaemia (MAHA). Incidence of cancer related MAHA remains unknown. Cancer-related MAHA are mainly observed in mucin-producer adenocarcinomas, such as gastric (half of reported cases) and breast cancer. We conducted a review of all original published cases of TM reported in breast cancer, and we specifically investigated BC-MAHA cases. A Medline search identified 158 MAHA cases including 118 BC-MAHA, and 40 drug-related MAHA. Most of BC-MAHA occur in disseminated cancers, mainly with medullar involvement, and/or bone metastasis. Patients typically suffer from poor general state, bone pain, and/or dyspnea. Laboratory abnormalities such as myelemia or erythromyelemia in peripheral blood are frequently observed. Incidence of coagulation disorders is increased, compared to other MAHA causes. BC-MAHA prognosis is dramatically poor. Treatments classically used in other MAHA causes, such as plasmapheresis or immunoglobulins, are inefficient. Urgent anti-neoplastic therapy may be the only effective treatment, associated to symptomatic therapies (transfusions, blood pressure control).

The Impact of Coagulopathy on Clinical Outcomes following Microsurgical Breast Reconstruction.

SUMMARY: Autologous breast reconstruction has evolved considerably from pedicled muscle-based approaches to microsurgical perforator-based techniques. Patients with documented coagulopathy, however, remain a particularly challenging population. The authors present their experience in microsurgical breast reconstruction in patients with coagulopathy and discuss their treatment protocol. A prospectively maintained database was queried for patients with coagulopathy who underwent microsurgical breast reconstruction between 2016 and 2019. Information regarding patient demographics, type of coagulopathy, and anticoagulation regimen were retrieved, and clinical outcomes were investigated. Nineteen patients who underwent 34 microsurgical breast reconstructions with free abdominal flaps were included in the study. The most common coagulopathy was factor V Leiden [n = 7 (38.6 percent)]. Nine patients (47.4 percent) developed thrombotic complications (the majority occurring intraoperatively); notably, arterial and venous thrombosis in four (21.1 percent) and two patients (10.5 percent), respectively. Postoperative thrombotic complications included pulmonary embolism [n = 2 (10.5 percent)] and flap congestion secondary to venous thrombosis [two flaps (5.9 percent)]. Only one flap loss was observed secondary to delayed venous thrombosis on postoperative day 6 (2.9 percent). The anticoagulation regimen in the majority of patients consisted of intraoperative intravenous administration of heparin (2000 U [bolus]) followed by a 5-day heparin infusion at 500 U/hour [n = 10 (52.6 percent)]. The high rate of thrombotic complications in patients with coagulopathy who underwent microsurgical breast reconstruction is contrasted by a low flap loss rate. Although coagulopathy is a risk factor for thrombotic complications, successful microsurgical breast reconstruction is still possible in the majority of patients.

Trends, characteristics, and outcomes of placenta accreta spectrum: a national study in the United States.

BACKGROUND: Although an infrequent occurrence, the placenta can adhere abnormally to the gravid uterus leading to significantly high maternal morbidity and mortality during cesarean delivery. Contemporary national statistics related to a morbidly adherent placenta, referred to as placenta accreta spectrum, are needed. OBJECTIVE: This study aimed to examine national trends, characteristics, and perioperative outcomes of women who underwent cesarean delivery for placenta accreta spectrum in the United States. STUDY DESIGN: This is a population-based retrospective, observational study querying the National Inpatient Sample. The study cohort included women who underwent cesarean delivery from October 2015 to December 2017 and had a diagnosis of placenta accreta spectrum. The main outcome measures were patient characteristics and surgical outcomes related to placenta accreta spectrum assessed by the generalized estimating equation on multivariable analysis. The temporal trend of placenta accreta spectrum was also assessed by linear segmented regression with log transformation. RESULTS: Of 2,727,477 cases who underwent cesarean delivery during the study period, 8030 (0.29%) had the diagnosis of placenta accreta spectrum. Placenta accreta was the most common diagnosis (n=6205, 0.23%), followed by percreta (n=1060, 0.04%) and increta (n=765, 0.03%). The number of placenta accreta spectrum cases increased by 2.1% every quarter year from 0.27% to 0.32% (P=.004). On multivariable analysis, (1) patient demographics (older age, tobacco use, recent diagnosis, higher comorbidity, and use of assisted reproductive technology), (2) pregnancy characteristics (placenta previa, previous cesarean delivery, breech presentation, and grand multiparity), and (3) hospital factors (urban teaching center and large bed capacity hospital) represented the independent characteristics related to placenta accreta spectrum (all, P<.05). The median gestational age at cesarean delivery was 36 weeks for placenta accreta and 34 weeks for both placenta increta and percreta vs 39 weeks for non-placenta accreta spectrum cases (P<.001). On multivariable analysis, cesarean delivery complicated by placenta accreta spectrum was associated with increased risk of any surgical morbidities (78.3% vs 10.6%), Centers for Disease Control and Prevention-defined severe maternal morbidity (60.3% vs 3.1%), hemorrhage (54.1% vs 3.9%), coagulopathy (5.3% vs 0.3%), shock (5.0% vs 0.1%), urinary tract injury (8.3% vs 0.2%), and death (0.25% vs 0.01%) compared with cesarean delivery without placenta accreta spectrum. When further analyzed by subtype, cesarean delivery for placenta increta and percreta was associated with higher likelihood of hysterectomy (0.4% for non-placenta accreta spectrum, 45.8% for accreta, 82.4% for increta, 78.3% for percreta; P<.001) and urinary tract injury (0.2% for non-placenta accreta spectrum, 5.2% for accreta, 11.8% for increta, 24.5% for percreta; P<.001). Moreover, women in the placenta increta and percreta groups had markedly increased risks of surgical mortality compared with those without placenta accreta spectrum (increta, odds ratio, 19.9; and percreta, odds ratio, 32.1). CONCLUSION: Patient characteristics and outcomes differ across the placenta accreta spectrum subtypes, and women with placenta increta and percreta have considerably high surgical morbidity and mortality risks. Notably, 1 in 313 women undergoing cesarean delivery had a diagnosis of placenta accreta spectrum by the end of 2017, and the incidence seems to be higher than reported in previous studies.

Malignant hyperthermia when dantrolene is not readily available.

BACKGROUND: Malignant hyperthermia is a rare but life-threatening pharmacogenetic muscle disorder characterized by abnormal hypermetabolic reactions and commonly triggered in susceptible individuals by volatile anesthetics or succinylcholine, or both. Unfortunately, the specific medicine dantrolene is not readily available in many countries including China. The aim of this study was to find the characteristics of malignant hyperthermia under the situation that dantrolene is not readily available. METHODS: The cases of malignant hyperthermia reported on the most commonly used databases in China from 1985 to 2020 were analyzed. The inclusion criteria were the MH episodes only related to anesthesia. The exclusion criteria were dubious MH episodes only caused by Ketamine administration or MH episodes irrelevant to anesthesia. Independent samples t-test and Pearson's chi-squared test were applied to assess the difference between the survived and death cases. RESULTS: Ninety-two cases of malignant hyperthermia reported on the most commonly used databases in China from 1985 to 2020 were analyzed. Median (IQR [range]) age was 18.5 (11.8-37.0 [0-70.0]) years. Compared with the survived cases, the death cases had higher maximum end-tidal partial pressure of CO2 (P = 0.033), the maximum arterial partial pressure of CO2 (P = 0.006), temperature first measured when the patient was first discovered abnormal (P = 0.012), and maximum temperature (P < 0.001). Besides, the death cases had less minimum pH (P < 0.001) and higher potassium (P < 0.001) and were more likely to have coagulation disorders (p = 0.018). Concerning treatment, cases used furosemide (P = 0.024), mannitol (P = 0.029), blood purification treatment (P = 0.017) had the advantage on the outcome. Creatine phosphokinase, myoglobin, and MB isoenzyme of creatine phosphokinase differed greatly among cases during the first week. 43 (46.7%) cases had congenital diseases. 12 (13.0%) cases were reported with abnormal laboratory test results or abnormal signs that are possibly relevant before anesthesia. CONCLUSIONS: In countries that dantrolene is not readily available, early warning, diagnosis, and prompt effective therapies are crucial for MH patients to survive.

A multicenter randomized controlled trial comparing administration of antithrombin III after liver resection (HiSCO-05 trial).

BACKGROUND: Posthepatectomy liver failure is a poor prognostic factor after hepatectomy. Various preventive treatments have been tried; however, there are no clinical trials that use posthepatectomy liver failure as the primary endpoint, and the clinical effects of posthepatectomy liver failure have not been fully verified. The aim of this study was to investigate whether administration of antithrombin III can prevent posthepatectomy liver failure in patients with coagulopathy after hepatectomy. This study also evaluated the safety of AT-III administration after hepatectomy. METHODS: The current study enrolled 141 patients diagnosed with coagulopathy after hepatectomy between October 2015 and September 2018 at 7 hospitals in Hiroshima, Japan (HiSCO group). Patients were randomized to undergo either administration of antithrombin III (n = 64) or non-administration (n = 77). The primary endpoint was the incidence of posthepatectomy liver failure. This randomized controlled trial was registered with the University Medical Information Network Clinical Trial Registry (UMIN000018852). RESULTS: Treatment for postoperative coagulopathy was performed safely without adverse events. The incidence of posthepatectomy liver failure was similar in both treatment groups (nonadministration of antithrombin III group, 28.5%, versus administration of antithrombin III group, 28.1%; P = .953) The rate of morbidity was higher in the administration group than the non-administrated group (17.2% vs 11.7%, P = .351). Following the multivariate analysis of the whole study group, body mass index ≥25, total bilirubin ≥1.5 mg/dL, and the disseminated intravascular coagulation score ≥5 postoperatively were the independent risk factors for posthepatectomy liver failure. CONCLUSION: This study showed that the administration of antithrombin III resulted in no significant difference in preventing posthepatectomy liver failure, possibly through suppressing coagulopathy.

Children and young adults hospitalized for severe COVID-19 exhibit thrombotic coagulopathy.

We report the clinical and laboratory coagulation characteristics of 27 pediatric and young adult patients (2 months to 21 years) treated for symptomatic COVID-19 at a children's hospital in the Bronx, New York, between March 1 and May 31, 2020. D-Dimer was > 0.5 µg/mL (upper limit of normal) in 25 (93%) patients at admission; 11 (41%) developed peak D-dimer > 5 µg/mL during admission. Seven (26%) patients developed venous thromboembolism: three with deep vein thrombosis and four with pulmonary embolism. Requirement of increased ventilatory support was a risk factor for thrombosis (P = 0.006). Three of eight (38%) patients on prophylactic anticoagulation developed thrombosis; however, no patients developed VTE on low-molecular-weight heparin prophylaxis titrated to anti-Xa level. Manifestation of COVID-19 disease was severe or critical in 16 (59%) patients. Four (15%) patients died of COVID-19 complications: all had comorbidities. Elevated D-dimer and increased VTE rate were observed in this young cohort, particularly in those with severe respiratory complications, suggesting thrombotic coagulopathy. More data are needed to guide thromboprophylaxis in this age group.

Coagulation Abnormalities in Light Chain Amyloidosis.

OBJECTIVE: To assess the prevalence of coagulation abnormalities in patients with systemic light chain (AL) amyloidosis and their association with disease-related characteristics, disease progression, and survival. PATIENTS AND METHODS: This is a retrospective study of patients with AL amyloidosis seen at Mayo Clinic, Rochester, Minnesota, from January 1, 2006, to December 31, 2015. We studied the association between abnormal coagulation parameters and baseline characteristics and their association with survival outcomes. RESULTS: The study included 411 patients. Abnormalities at diagnosis included prolonged clotting times and coagulation factor deficiencies; prolonged prothrombin time (PT) and factor X (FX) deficiency were found in 19% (73 of 390) and 43% (177 of 411) of patients, respectively. The FX deficiency was associated with higher Mayo stage, involvement of more than 1 organ, liver and cardiac involvement, and greater than 10% bone marrow plasma cells. On univariate analysis, the risk for disease progression or death was higher in patients with abnormal values for PT and factor V, factor VII (FVII), FX, and factor XII compared with those with normal values. Prolonged PT and FVII and FX deficiencies were independent predictors of death after adjusting for Mayo stage and more than 1 organ involvement. Only 106 patients had repeat testing after treatment; no clear relationship was found between treatment response and changes in coagulation parameters. CONCLUSION: Coagulation abnormalities occur in a significant proportion of patients with AL amyloidosis and are associated with advanced disease and inferior outcomes. Larger studies are needed to establish whether a relationship exists between treatment response and improvement in individual parameters.

Acute thrombotic manifestations of coronavirus disease 2019 infection: Experience at a large New York City health care system.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is a novel coronavirus that has typically resulted in upper respiratory symptoms. However, we have encountered acute arterial and venous thrombotic events after COVID-19 infection. Managing acute thrombotic events from the novel virus has presented unprecedented challenges during the COVID-19 pandemic. In our study, we have highlighted the unique treatment required for these patients and discussed the role of anticoagulation for patients diagnosed with COVID-19. METHODS: The data from 21 patients with laboratory-confirmed COVID-19 disease and acute venous or arterial thrombosis were collected. The demographics, comorbidities, home medications, laboratory markers, and outcomes were analyzed. The primary postoperative outcome of interest was mortality, and the secondary outcomes were primary patency and morbidity. To assess for significance, a univariate analysis was performed using the Pearson χ2 and Fisher exact tests for categorical variables and the Student t test for continuous variables. RESULTS: A total of 21 patients with acute thrombotic events met our inclusion and exclusion criteria. Most cases were acute arterial events (76.2%), with the remainder venous cases (23.8%). The average age for all patients was 64.6 years, and 52.4% were male. The most prevalent comorbidity in the group was hypertension (81.0%). Several markers were markedly abnormal in both arterial and venous cases, including an elevated neutrophil/lymphocyte ratio (8.8) and D-dimer level (4.9 µg/mL). Operative intervention included percutaneous angiography in 25.00% of patients and open surgical embolectomy in 23.8%. Most of the patients who had undergone arterial intervention had developed a postoperative complication (53.9%) compared with a 0% complication rate after venous interventions. Acute kidney injury on admission was a factor in 75.0% of those who died vs 18.2% in the survivors (P = .04). CONCLUSIONS: We have described our experience in the epicenter of the pandemic of 21 patients who had experienced major thrombotic events from infection with COVID-19. The findings from our cohort have highlighted the need for increased awareness of the vascular manifestations of COVID-19 and the important role of anticoagulation for these patients. More data are urgently needed to optimize treatment and prevent further vascular complications of COVID-19 infections.

Predicting Postoperative Mortality After Metastatic Intraspinal Neoplasm Excision: Development of a Machine-Learning Approach.

OBJECTIVE: Mortality following surgical resection of spinal tumors is a devastating outcome. Naïve Bayes machine learning algorithms may be leveraged in surgical planning to predict mortality. In this investigation, we use a Naïve Bayes classification algorithm to predict mortality following spinal tumor excision within 30 days of surgery. METHODS: Patients who underwent laminectomies between 2006 and 2018 for excisions of intraspinal neoplasms were selected from the National Surgical Quality Initiative Program. Naïve Bayes classifier analysis was conducted in Python. The area under the receiver operating curve (AUC) was calculated to evaluate the classifier's ability to predict mortality within 30 days of surgery. Multivariable logistic regression analysis was performed in R to identify risk factors for 30-day postoperative mortality. RESULTS: In total, 2094 spine tumor surgery patients were included in the study. The 30-day mortality rate was 5.16%. The classifier yielded an AUC of 0.898, which exceeds the predictive capacity of the National Surgical Quality Initiative Program mortality probability calculator's AUC of 0.722 (P < 0.0001). The multivariable regression indicated that smoking history, chronic obstructive pulmonary disease, disseminated cancer, bleeding disorder history, dyspnea, and low albumin levels were strongly associated with 30-day mortality. CONCLUSIONS: The Naïve Bayes classifier may be used to predict 30-day mortality for patients undergoing spine tumor excisions, with an increasing degree of accuracy as the model better performs by learning continuously from the input patient data. Patient outcomes can be improved by identifying high-risk populations early using the algorithm and applying that data to inform preoperative decision making, as well as patient selection and education.

Severe Rare Bleeding Disorders: A Single-center Experience.

Although rare bleeding disorders (RBDs) are not common diseases, they are important for life-threatening bleedings and prophylaxis approaches, especially in severe forms. In this retrospective study, the authors have analyzed data from children with severe RBDs who were examined at the center over a period of 10 years to describe the distribution, clinical features, treatment patterns, and outcome of severe RBDs in patients. Data from all children (age under 18 y) with RBDs who were examined in the center between 2005 and 2015 were retrospectively reviewed. In total, 12 patients were included in the study. Four of the cases had factor (F) VII (33.3%), 6 had FX (50%), 1 had FXIII (8.3%), and 1 had fibrinogen deficiency (8.3%). Of the 12 children with severe RBDs, 8 (67%) experienced at least 1 major bleeding. Prophylaxis was applied to 10 patients. In conclusion, RBDs are more common in our country because of the high parental consanguinity rates. So, it is necessary to raise public awareness about the risks of consanguineous marriages and increase access to genetic counseling and testing facilities. Delayed diagnosis and lack of adequate prophylactic replacement therapy are the most important risk factors that increase life-threatening bleeding.