Lower Prothrombin Time Research Articles

Sex-Specific Differences in Sepsis Development in Polytrauma Patients Undergoing Stand-Alone Definitive Surgery

Background and Objectives: In the triaging of polytrauma, patients with less severe injuries and lower somatic stress often undergo isolated definitive orthopedic surgery without damage-control procedures. Adverse events, particularly sepsis, should be minimized. We aimed to identify sex-specific predictors for sepsis in polytrauma patients undergoing stand-alone definitive surgery. Materials and Methods: Our institutional trauma database, containing data from 3653 patients between 1996 and 2022, was filtered for polytrauma patients who underwent definitive surgery, were aged ≥16 years, and had an Injury Severity Score (ISS) ≥16. Injury and physiological parameters were documented upon admission, as well as on the first and second days thereafter. Surgical data were also recorded. All factors were evaluated for their association with sepsis development. Results: Among the 276 patients (71.1% male; mean age 45.0 years, 95% confidence interval 42.7–47.2 years; median ISS of 27, interquartile range, 20–34), the rate of sepsis was 13.8% (n = 38), with a higher incidence in males (16.7%; n = 33) than in females (6.8%; n = 5) (p = 0.026). Head and thorax injuries were more common and severe in septic males, with thorax injuries being predictive. Male patients who developed sepsis also had a higher ISS, an increased heart rate (HR), and lower pH at admission, with ISS and HR being predictive of sepsis. On the first day post-admission, septic males showed higher Base Excess and lower Prothrombin Time. Lactate levels were elevated on both the first and second days post-admission. Surgical predictors for males included less primary extremity surgeries and later secondary spine surgeries. Conclusions: Sepsis is more common in males undergoing stand-alone definitive surgery. Several factors, particularly acidosis and coagulopathy, are associated with this phenomenon. Fewer primary extremity surgeries and delayed secondary spine surgeries were also linked to a higher sepsis risk in males. These findings may help identify patients eligible for stand-alone definitive surgery and underscore the need for more data on female polytrauma patients.

Risk factors for intrahepatic venovenous shunt in patients with cirrhosis and its impact on hepatic venous pressure gradient

Objective: To evaluate the factors affecting the incidence of intrahepatic venovenous shunt (IVVS) in patients with cirrhosis and its impact on hepatic venous pressure gradient (HVPG). Methods: A retrospective analysis was performed on the data of patients with liver cirrhosis who received HVPG measurement in Nanjing Drum Tower Hospital from April 2013 to March 2022. Univariate and multivariate regression analyses were used to investigate the incidence rate and risk factors of IVVS and its impact on HVPG. The t-test and rank-sum test were used for the measurement data, and the χ2 test was used for the count data. Results: A total of 242 cases with cirrhosis were included in the statistical analysis, including 54 (22.3%) with IVVS and 188 (77.7%) without IVVS. There was a statistically significant difference (P<0.05) in prothrombin time (PT), HVPG, and splenectomy history between the two groups of patients' baseline data (all P<0.05). The multiple logistic regression analysis results showed that PT was an independent risk factor for the occurrence of IVVS (P<0.05), and patients combined with IVVS had lower HVPG values [(17.58±5.57) mmHg vs. (11.92±5.38) mmHg, 1 mmHg=0.133 kPa; t=6.623, P<0.001]. Conclusions: Patients with liver cirrhosis have a high incidence rate of IVVS, which is closely associated with a low prothrombin time. Additionally, patients combined with IVVS have low HVPG values, which affect its accuracy.

Predictive haemostatic biomarkers and transfusion efficacy, insights from fresh frozen plasma use in surgical patients, preliminary results

The aim of the study was to examine various haemostasis values to identify the most relevant biological indicators for detecting significant haemorrhage, to determine the effectiveness of fresh frozen plasma (FFP) transfusion. Our findings suggest that a low prothrombin time, elevated Von Willebrand Antigen, increased plasma fibrinogen, and reduced Ca2 + levels are associated with challenges in achieving proper haemostasis. However, measurements of factors II, V, VII, VIII, IX, X, XI, XIII, protein C, and protein S do not appear to be linked to difficulties in achieving adequate haemostasis. Additionally, the administration of FFP appears to impact factors V, VII, X, and II.Trial registration EudraCT number: 2019-002898-64.

Changes of coagulation function and platelet parameters in preeclampsia and their correlation with pregnancy outcomes.

Preeclampsia (PE) is a severe pregnancy complication characterized by significant alterations in coagulation function. This study aims to analyze the correlation between coagulation function, platelet parameters, and pregnancy outcomes in PE patients. Clinical data, along with blood and urine samples, were collected from 168 PE patients and 128 healthy pregnant women. General demographic and laboratory testing data were recorded, and maternal and fetal outcomes were followed up. Data were analyzed using Kaplan-Meier and logistic regression analyses. In mild PE patients, thrombin time (p=.000), platelet distribution width (PDW) (p=.000), and clot formation time (p=.000) were increased, while prothrombin time (p=.000) and fibrinogen (p=.045) were reduced. With increasing PE severity, prothrombin time (p=.000), platelet count (PLT) (p=.000), mean platelet volume (MPV) (p=.000), plateletcrit (p=.000), maximum amplitude (MA) (p=.000), and coagulation index (p=.001) decreased, whereas activated partial thromboplastin time (APTT) (p=.000), thrombin time (p=.002), D-dimer (p=.026), and PDW (p=.000) increased. Lower prothrombin time (p=.048), PLT (p=.004), and coagulation index (p=.026) or higher APTT (p=.032), thrombin time (p=.044), D-dimer (p=.023), and PDW (p=.016) were associated with a higher risk of poor pregnancy outcomes. Thrombin time was identified as an independent risk factor (p=.025, OR=2.918, 95% CI: 1.145-7.436), whereas gestational age was an independent protective factor (p=.000, OR=0.244, 95% CI: 0.151-0.395). This study demonstrates that specific coagulation and platelet parameters are significantly associated with PE severity and adverse pregnancy outcomes. These findings highlight the importance of monitoring coagulation function in PE patients to improve clinical management and outcomes.

Budd–Chiari syndrome at the cirrhosis stage: A case report from Campus Teaching Hospital of Lome-Togo

Abstract Budd–Chiari syndrome is a rare disease and is a significant cause of cirrhosis. We report a clinical case of a 23-year-old patient, highlighting the diagnostic and therapeutic difficulties of Budd–Chiari syndrome at the cirrhosis stage in a country with limited resources such as Togo. A 23-year-old, nonalcoholic, patient who tested negative for hepatitis B and C virus presented with progressive abdominal distension. Examination revealed grade 2 ascites and soft, painless, cold, bucketing edema of the lower limbs rising to the knees. The calculated serum-ascites albumin gradient was 12 g/L. Abdominal ultrasound and abdominal computed tomography indicated the presence of thrombosis of the inferior vena cava, which ascended to the hepatic veins. Biological signs of hepatocellular insufficiency such as a low prothrombin time (56%) and low serum albumin levels (22 g/L) with a beta-gamma block were also found. Upper gastrointestinal endoscopy revealed grade 3 esophageal varices with red signs. The diagnosis of Budd–Chiari syndrome at the stage of cirrhosis classified as Child–Pugh score 9 decompensated in the ascitic mode, complicated by grade 3 esophageal varices with red signs, was retained. Due to the inadequacy of the technical facilities in Togo, and also because of financial difficulties, aetiological work-up was not carried out. Anticoagulant treatment with rivaroxaban 10 mg/day has been instituted. The clinical outcomes were poor with gastrointestinal bleeding, stage 3 hepatic encephalopathy, and death on the 46th day. The prognosis of Budd–Chiari syndrome is poor in resource-limited countries such as Togo. It depends on the quality of care, not only of the etiology but also of the complications of cirrhosis.

Glycated serum protein is independently associated with progressive infarction in patients with acute ischemic stroke.

This study investigated the relationship between glycated serum protein (GSP) and progressive infarction (PI). From April 2017 to December 2020, we recruited 477 patients within 48 hours after the onset of acute ischemic stroke into this case-control study. Demographic characteristics, clinical information, and laboratory and neuroimaging data were recorded after admission. PI occurred in 144 (30.8%) patients. Patients with PI had higher initial National Institute of Health Stroke Scale (NIHSS) scores, higher discharge NIHSS scores, higher modified Rankin scale scores at 3 months after onset, higher GSP levels, lower prothrombin times, and lower creatinine levels than patients without PI. The likelihood of PI increased with increases in the GSP quartile. Multiple regression analysis revealed that high GSP levels (>2.14 mmol/L) were independently associated with PI. Subgroup analyses identified high GSP levels as an independent predictor of PI in patients with large artery atherosclerosis (third quartile: odds ratio [OR] = 3.793; 95% confidence interval [CI] = 1.555-9.250; fourth quartile: OR = 2.675; 95% CI = 1.056-6.776) and anterior circulation small vessel occlusion (fourth quartile: OR = 13.859; 95% CI = 2.024-94.885). GSP might be an independent predictor for PI in certain patients with acute ischemic stroke.

Alagille syndrome and liver: an adult case report

BackgroundAlagille syndrome is a rare autosomal-dominant disorder, representing 10 to 15% of the causes of neonatal cholestasis with no gender predominance. The diagnosis is based on the association of liver, heart, eye, skeleton abnormalities, and characteristic facial appearance.Case presentationAn 18-year-old male patient, with a family history of benign recurrent intrahepatic cholestasis in a brother, was diagnosed at birth with bile duct paucity. He consulted in adulthood for cholestatic jaundice and pruritus. Physical exam found cutaneous jaundice, particular face, skeletal abnormality of fingers, posterior embryotoxon, and splenomegaly. An echocardiogram found cardiovascular abnormalities. The diagnosis of Alagille syndrome was made in front of five major criteria. A liver biopsy revealed a cirrhosis liver. Upper gastrointestinal endoscopy revealed grade II esophageal varices of portal hypertension. Laboratory tests revealed bicytopenia related to hypersplenism, hypoferritinemia, cytolysis with cholestasis, high bilirubin levels, low prothrombin time, hypoalbuminemia, decreased factor V activity, and hypocholesterolemia. The patient had vitamin K supplementation and was put on ursodeoxycholic acid, propranolol for the liver disease, a high protein hypercaloric diet for malnutrition, vitamin D supplementation and bisphosphonate for the osteoporosis, therapeutic abstention with monitoring for the asymptomatic cardiac disease. After a year of treatment, the patient had an overall health status improvement. Abdominal ultrasound found liver nodules. A biliary MRI showed a multinodular liver. The complement by CT hepatic angiography did not show any nodules while the MRI angiography revealed multiple dysplastic nodules. A liver biopsy was performed and found regenerative nodules.ConclusionThe treatment of Alagille syndrome is based on managing the cholestasis and its complications, especially pruritus because it can have a significant impact on quality of life. Due to the complexity of presentation and multi-organ involvement, management of cases with Alagille syndrome should be done by a multidisciplinary team. Liver disease is responsible for morbidity while cardiac disease is a mortality risk factor in this population.

Paediatric COVID-19 Outcomes: Haematology Parameters, Mortality Rates, and Hospitalization Duration.

The global COVID-19 pandemic has strained healthcare systems around the globe, necessitating extensive research into the variables that affect patient outcomes. This study examines the relationships between key haematology parameters, duration of hospital stay (LOS), and mortality rates in COVID-19 cases in paediatric patients. Researchers analyse relationships between independent variables (COVID-19 status, age, sex) and dependent variables (mortality, LOS, coagulation parameters, WBC count, RBC parameters) using multivariate regression models. Although the R-square values (0.6-3.7%) indicate limited explanatory power, coefficients with statistical significance establish the impact of independent variables on outcomes. Age emerges as a crucial predictor of mortality; the mortality rate decreases by 1.768% per age group. Both COVID-19 status and age have an inverse relationship with length of stay, emphasising the milder hospitalisation of children. Platelet counts decline with age and male gender, potentially revealing the influence of COVID-19 on haematological markers. There are significant correlations between COVID-19 status, age, gender and coagulation measures. Lower prothrombin time and D-dimer concentrations in elder COVID-19 patients are indicative of distinct coagulation profiles. WBC and RBC parameters exhibit correlations with variables: COVID-19-positive patients have lower WBC counts, whereas male COVID-19-positive patients have higher RBC counts. In addition, correlations exist between independent variables and the red cell distribution width, mean corpuscular volume, and mean corpuscular haemoglobin. However, there is no correlation between mean corpuscular haemoglobin concentration and outcomes, indicating complex interactions between haematological markers and outcomes. In essence, this study underlines the importance of age in COVID-19 mortality, provides novel insights into platelet counts, and emphasises the complexity of the relationships between haematological parameters and disease outcomes.

Coagulation Abnormalities in Severe Scrub Typhus and Their Association with Complications.

To describe coagulation abnormalities and their association with complications in patients with severe scrub typhus. A cohort study was conducted among all patients of severe scrub typhus [immunoglobulin M (IgM) positive] who reported to this facility from 1st August 2019 to 31st July 2020 and met our inclusion criteria. We estimated the incidence of severe thrombocytopenia (&lt;50,000/µL) and overt disseminated intravascular coagulation (DIC) (DIC score of ≥5). We determined the association [risk (RR) ratios] of these abnormalities with complications of scrub typhus, namely-septic shock, multiple organ dysfunction syndrome (MODS), and septic shock with MODS. In total, 71 patients were studied with a mean age of 50 ± 15.5 years, of which 45 (63.4%) were females. On presentation, fever 70 (98.5%), myalgias 22 (31.0%), loose stools 13(18.3%), cough, vomiting, headache 11 (15.5%), altered sensorium 10(14.1%), and pain abdomen 9 (12.7%) were main symptoms. On examination, hypotension 31 (43.7%), eschar 25 (35.2%), icterus 17 (23.9%), and rash 16 (22.5%) were noted. The d-dimer (&gt;0.5 µg/mL) levels were increased in all (100%) patients. Thrombocytopenia (91.5%) was the commonest hematological abnormality and 31 (43.6%) of them had severe thrombocytopenia, 25 (35.2%) patients had low fibrinogen levels (&lt;200 mg/dL) and prothrombin time (PT &gt;16.7 seconds) was prolonged in 20 (28.1%). A total of 42 (59.1%) patients developed MODS, 33 (46.4%) developed septic shock, 24 (33.8 %) had MODS with septic shock, 17 (23.9%) developed overt DIC, and eight (11.2%) died. Severe thrombocytopenia (p = -0.028) and overt DIC (p = 0.045) were significantly associated with septic shock development. In the patients admitted with severe scrub typhus; thrombocytopenia was the commonest hematological abnormality. The development of septic shock was significantly associated with severe thrombocytopenia and overt DIC.

Case Report: Hyperemesis gravidarum, high transaminases level and prolonged prothrombin time: is it an acute liver injury?

Background: Hyperemesis gravidarum (HG) is a severe form of nausea and vomiting in the first trimester of pregnancy. It is considered a benign condition, but severe complications, fortunately rare, have been reported. Frequently, this condition is associated with a perturbed liver function tests, which remains without severe consequences. The clinical presentation may be suggestive of an acute liver injury (ALI), especially as the end of the first trimester approaches, pregnancy specific and non-specific liver diseases should be considered. Case: A 28-year-old primigravida, 14-week pregnant woman affected by hyperemesis gravidarum, developed high transaminases level and spontaneously low prothrombin time (PT) ratio. An ALI was suspected as transaminases were very high and our patient was at the end of the first trimester. An exhaustive etiological work-up was negative. In the second line, the factor V assay was conducted, which showed a normal activity, and the vitamin K level was low. We therefore concluded that it was hyperhemesis gravidarum complicated by fluid and electrolyte disorders and vitamin K deficiency. She had parenteral rehydration and a proton pump inhibitor. She received intravenous vitamin K 10 mg daily for three days. The outcome was excellent without any maternal or fetal impact. Conclusion: Hyperhemesis gravidarum is a common condition in the first trimester of pregnancy that usually has a favourable outcome. However, it is important to be attentive to possible complications, including vitamin K deficiency with its maternal and fetal consequences. On the other hand, in case of major disturbance of the liver function tests, we should not overlook acute liver injury and should not hesitate to initiate an adequate etilogical investigation.

FEATURE OF CORONAVIRUS DISEASE IN PATIENTS WITH ARTERIAL HYPERTENSION AND CHRONIC OBSTRUCTIVE PULMONARY DISEASE

Relevance. The combination of coronavirus disease (COVID-19) with arterial hypertension (AH) and chronic obstructive pulmonary disease (COPD) is associated with decreased saturation, general clinical signs of inflammation, coagulopathies and worsens the course and prognosis of the disease. That is why it is important to identify all possible risk factors for complicated pathology for the prevention and timely treatment of this category of patients.&#x0D; Objective: to study the feature of coronavirus disease in patients with AH and COPD.&#x0D; Materials and methods. A total of 101 patients aged 30 to 80 years with COVID-19 were examined. The first group included 26 people with COVID-19 and COPD (GOLD 2, groups A, B), and the second group included 75 patients with COVID-19 and AH (I-III stages, 1-3 grade). The parameters of the general blood test, biochemical blood test, coagulation testing, D-dimer, C-reactive protein (CRP), and ferritin in the blood were studied.&#x0D; Results. Patients with COVID-19 and COPD had higher erythrocyte sedimentation rate (p&gt; 0,05) and leukocytosis (p &lt;0,05), as well as increased concentrations of creatinine (p &lt; 0,05) and urea (p &lt; 0,01) that indicates the presence of renal pathology. Patients with COVID-19 and AH had significantly lower prothrombin time (p &lt; 0,05) and international normalized ratio (p &lt; 0,05), as well as a higher prothrombin index (p &lt;0,05), which indicates the benefits of the procoagulant properties of blood in this category of patients. The level of D-dimer (p&gt; 0,05), CRP (p&gt; 0,05), and ferritin (p &lt;0,05) was higher in patients of the 2nd group, which indicates a more severe course of coronavirus disease, as well as more high risks of thromboembolic complications.&#x0D; Conclusions. The features of coronavirus disease in patients with hypertension indicate a more severe course of the pathology and a high risk of thromboembolic complications, as evidenced by the procoagulant properties of blood and a high level of D-dimer, CRP, and ferritin. According to the data of clinical and laboratory examinations in patients with COPD and COVID-19, there was a significant increase in creatinine, urea, and leukocytosis, which may indicate the addition of renal pathology.

Variations of prothrombin time among ABO blood groups in healthy adults

Background and objectives: ABO blood group distribution defers with racial and geographic variations. They are related with diseases like cardiovascular diseases, cerebral thromboembolism. Prothrombin time may varies among ABO blood group system which may increase the future risk of thrombosis. The present study is to assess prothrombin time among ABO blood groups in healthy adults. Materials and methods: A prospective type of analytical cross-sectional study was conducted in the Department of Physiology, Dhaka Medical College, Dhaka from July 2019 to June 2020. After obtaining ethical clearance, a total 190 healthy adults were selected from different areas of Dhaka city based on inclusion and exclusion criteria, with age ranging from 18-45 years. The subjects were interviewed and detailed history regarding personal, family, medical and drug were taken. Prior to sample collection, informed written consent was taken from the participants. Individuals of blood group A was selected as group A, blood group B as group B, blood group AB as group AB and blood group O as group O. Prothrombin time was measured in the Department of Hematology and BMT Unit, Dhaka Medical College Hospital, Dhaka. Blood grouping was done in the Department of Physiology, Dhaka Medical College, Dhaka.For statistical analysis, One way ANOVA followed by Bonferroni test were considered using SPSS 25.0 version. Results: In this study, blood group B was most common (33.2%). Prothrombin time was lower in blood group A (12.93±0.30 sec), B (12.92±0.61 sec), AB (12.98±0.54 sec) than blood group O (13.08±0.39 sec) which was not statistically significant. Conclusion: It can be concluded that blood group A, B, AB individuals may have more chance of thrombosis due to lower prothrombin time than blood group O individuals.

Enhanced liver fibrosis score as a predictive marker for hepatocellular carcinoma development after hepatitis C virus eradication.

Advanced liver fibrosis is the most important risk factor for hepatocellular carcinoma (HCC) development after achieving sustained virological response (SVR) by direct-acting antiviral (DAA) treatment in patients with chronic hepatitis C. Wisteria floribunda agglutinin-positive Mac-2-binding protein (M2BPGi), enhanced liver fibrosis (ELF) score, type IV collagen and fibrosis-4 (FIB-4) index have been reported as non-invasive biomarkers for liver fibrosis. In the present study, the possibility of using fibrosis biomarkers and other parameters to predict the development of HCC was evaluated. A total of 743 patients infected with hepatitis C virus who achieved SVR by using DAA were retrospectively enrolled. Of these, 122 patients whose blood samples were stored were selected. The aforementioned four fibrosis biomarkers were analyzed at baseline, at the end of treatment (EOT) and at post-treatment week 24 (PTW24). Tumor markers and laboratory tests were also analyzed. The baseline/EOT/PTW24 values for each fibrosis biomarker were as follows: ELF score: 11.5±1.2/10.8±1.1/10.4±1.0; type IV collagen: 213±85/190±67/174±55 ng/ml; M2BPGi: 4.8±3.5/2.7±2.0/2.2±1.8; and FIB-4 index: 5.31±3.82/4.36± 2.79/4.24±3.09. Of the 122 patients, 23 developed HCC. A high baseline ELF score (P=0.0264), PTW24 ELF score (P=0.0003), PTW24 α-fetoprotein level (P=0.0133), baseline FIB-4 index (P=0.0451) and low baseline prothrombin time (P=0.0455) were risk factors for HCC development based on univariate analyses. Based on the multivariate analysis, a high PTW24 ELF score was the only risk factor for HCC development (P=0.0035). The ELF score after DAA therapy was strongly associated with HCC development; therefore, it may be a useful marker for predicting HCC.

Prognostic Impact of Lymphocyte–C-Reactive Protein Ratio in Patients Who Underwent Surgical Resection for Hepatocellular Carcinoma

BackgroundSystemic inflammation-related factors, either independently or in combination, are recognized as prognostic factors for various cancers. The ratio of lymphocyte count to C-reactive protein concentration (lymphocyte–CRP ratio; LCR) is a recently identified prognostic marker for several cancers. Here, we examined the prognostic value of the LCR in patients with hepatocellular carcinoma (HCC). MethodsThis was a single-center retrospective study of patients who underwent surgical resection for HCC between 2004 and 2017. Patients were divided into high- and low-LCR status groups, and the relationships between LCR status, prognosis, and other clinicopathological characteristics were analyzed. ResultsA total of 454 patients with HCC were enrolled and assigned to the high- (n=245) or low- (n=209) LCR groups. Compared with the high-LCR group, patients in the low-LCR group had a significantly lower serum albumin level (median 4.1 vs. 3.9 g/dL, P <0.0001), lower platelet count (median 14.0 vs. 12.0 ×104/μL, P=0.0468), lower prothrombin time (median 93.2 vs. 89.6 %, P=0.0006), and larger tumor size (median 2.3 vs. 2.5 cm, P=0.0056). Patients with low-LCR status had significantly worse outcomes of overall survival and disease-free survival than patients with high-LCR status (P=0.0003 and P=0.0069, respectively). Low-LCR status was significantly associated with worse overall survival in multivariate analysis (hazard ratio 1.57, 95% confidence interval 1.14–2.17, P=0.0058). ConclusionsLow-LCR status may predict worse outcomes in patients with HCC. Measurement of LCR is routine and can easily be applied for risk stratification in the assessment of patients with HCC.

Hepatic Complications in Children with Sickle Cell Anemia Are Rare but Potentially Lethal

▪Background: Liver disease in sickle cell anemia (SCA) can be due to several pathophysiological processes, including acute or chronic ischemia/reperfusion injuries, increased bilirubin load in bile secondary to chronic hemolysis, iron overload, viruses, and autoimmune disorders. Severe hepatic impairment is rarely reported in children but may be underdiagnosed and insufficiently treated. We report 12 cases, highlighting the need for urgent recognition and treatment of SCA hepatic dysfunction.Patients and methods: We performed a single-center retrospective cohort study in our French Teaching Hospital SCA Referral Centre. We analyzed the medical files of 1532 children hospitalized between 2009 and 2017 and identified 12 patients aged 0.6 to 18 years (11 HbSS, 1 HbS/beta 0thalassemic) with severe liver dysfunction.Results: Acute hepatic crisis developed in 7 patients, age 4.5 to 17 years. Previous treatments were hydroxycarbamide (HC) (n=5), and monthly red blood cell (RBC) exchange transfusions coupled with deferasirox (n=3, including 1 case of documented overdosage). Bilirubin was 31-139 µM, more than half conjugated in 4/7 cases. ALT was 39-1305 IU. Three patients had liver failure at presentation, with encephalopathy, low prothrombin time (14-29 %) and hyperammoniemia (240-338 µM). RBC exchange transfusion was emergently performed in 5/7 patients, and a single RBC transfusion was performed in one patient without liver failure. All 6 patients recovered. One patient received an urgent liver transplantation without exchange transfusion, and died during surgery.Three patients, aged 3 to 14 years, developed a cholangiopathy, evidenced on MR-cholangiography. Additional factors were autoimmunity in one patient with treated autoimmune hepatitis and positive p-ANCA, and a heterozygous mutation in a gene involved in bile formation (MDR3) in another patient. Two patients needed a temporary biliary drainage to control infectious cholangitis, and one of them underwent subsequent biliary-digestive anastomosis followed with liver transplantation, with a good result (follow-up 2.5 years).Another patient, aged 18 years had autoimmune hepatitis, diagnosed on high ALT, positive autoantibodies and liver biopsy. He was successfully treated with prednisone and azathioprine.A 7-month-old child developed acute hepatic sequestration associating major hepatomegaly and drop in hemoglobin level (48 g/l), and received RBC transfusion with a good evolution.Discussion: Acute hepatic crisis is rare but potentially devastating and may develop in SCA patients undergoing chronic RBC exchange transfusions, even with HbS < 20%. It is assumed to be due to acute extensive sickling in liver sinusoids resulting in hepatocyte ischemia. The liver involvement should urgently be recognized, when hepatomegaly, conjugated bilirubin and/or high ALT are present. RBC exchange transfusion must be performed in emergency, and is often efficient to reverse liver failure. Liver transplantation does not appear to be a good option, as observed in our experience. Additional infectious or toxic factors may be involved and should be looked for.Cholangiopathies are probably caused by chronic ischemia of the bile ducts, maybe favored by a genetic or immunological predisposition. The main complication is chronic infectious cholangitis. Liver transplantation may be discussed. Autoimmune liver diseases seem to be more frequent in SCA patients. Liver biopsy when needed should only be performed after RBC exchange transfusion. Steroid treatment should be associated with intensification of SCA treatment (HC or chronic RBC transfusion). Liver sequestration is exceptional. DisclosuresDe Montalembert:Novartis: Consultancy, Honoraria, Research Funding; Addmedica: Consultancy, Honoraria, Research Funding. Brousse:Add Medica: Membership on an entity's Board of Directors or advisory committees.

MO324A RARE CASE OF GRANULOMATOSIS WITH POLYANGIITIS ASSOCIATED WITH FACTOR II DEFICIENCY

Abstract Background and Aims Granulomatosis with polyangiitis (GPA) (formerly called Wegener's) is a systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins. The kidney damage is severe. We report an observation of a young patient whose exploration of rapidly progressive renal failure revealed GPA with an original association with factor II deficiency. Method This a case report of a patient who was admitted to the nephrology, dialysis and transplantation in the sahloul university hospital in Tunisia. She was transferred from the regional hospital of sidi bouzid. Results This a case of a 19-year-old patient. She had the only a history of allergic rhinitis. She presented recently epistaxis and headache. The explorations reported a kidney failure with level of creatinine at 5.6 mg/dl. On examination: The blood pressure was normal (120/70 mmHg), she had signs of acute sinusitis and edema in both lower limbs and she developed anuria. For the biology: creatinine = 11.3 mg/dl (clearance = 4.6 ml/min), urea = 50 mmol/l, Proteinuria = 2.5 g/24h and Hematuria = 100/mm3. The cANCA type PR3 was positive. The diagnosis of rapidly progressive glomerulonephritis (RPGN) was suspected. She had microcytic hypochromic anemia of 6.9 g/dL and a low Prothrombin time (PT) of 54%. The exploration concluded to an isolated Factor II deficiency (59%). The Ear, Nose and throat (ENT) examination revealed nasal inflammation, sinusitis and nasal crusting. The kidney ultrasound showed two kidneys of normal size. The computed tomography showed bilateral maxillary sinusitis and there were no intra-alveolar hemorrhage. The Kidney biopsy could not be performed initially due to the risk of bleeding. On hospitalization, the patient presented a generalized tonic clonic seizures. The exploration by a cerebral Magnetic resonance imaging (MRI) showed signs of cerebral vasculitis and the electroencephalography (EEG) showed no focal abnormalities. The association of ENT involvement, the cerebrovascular disease, the rapid evolution of the creatinine, the positivity of the cANCA, the diagnosis of granulomatosis with polyangiitis was established. The therapeutic management was to start symptomatic treatment: diuretics, antibiotic therapy, transfusions, hemodialysis initially and antiepileptic therapy. The immunosuppressive treatment was based on pulses of methylprednisolone followed by full dose corticosteroid therapy, cyclophosphamide intravenous and sessions of plasma exchange. The evolution was characterized by clinical and biological improvement with creatinine = 1.3 mg/dl, a PT at 100% and a factor II at 70 % stable after stopping the plasma exchange in favor of the immunological cause of this deficit. Conclusion The early diagnosis and treatment of RPGN is important even if the biopsy cannot be performed. We reported a rare association between granulomatosis with polyangiitis and factor II deficiency. The normalization of the factor II after the treatment is in favor of the immunological cause of this deficit.

Clinical characteristics of sarcopenia in patients with alcoholic liver cirrhosis.

Background and AimSarcopenia frequently develops in patient with liver cirrhosis (LC). Ethanol reduces muscle protein synthesis and accelerates proteolysis. However, the relationship between heavy alcohol consumption and sarcopenia remains controversial. This study aimed to investigate the characteristics and prevalence of sarcopenia among patients with alcoholic LC (ALC) in real‐world clinical settings.MethodsThis cross‐sectional study included 181 patients with LC. Heavy alcohol consumption was defined as >60 g/day. Sarcopenia was diagnosed according to the Japan Society of Hepatology criteria.ResultsAmong the 181 patients, 64 (35.4%) were diagnosed with ALC. Patients with ALC were younger (median, 61.5 vs 72.0 years; P < 0.001) and had a lower prevalence of sarcopenia (18.8 vs 32.5%; P = 0.048) than those with non‐ALC. Conversely, the former had a higher prevalence of Child–Pugh class B/C (P = 0.015), higher total bilirubin (P = 0.017), and lower prothrombin time (P < 0.001) than the latter. The prevalence of sarcopenia increased alongside advancing age in patients with ALC (P = 0.007). Multivariate analysis identified older age (but not disease stage/liver function reserve and alcohol consumption) as an independent factor associated with sarcopenia (P = 0.002) in patients with ALC.ConclusionPatients with ALC were younger and had a lower prevalence of sarcopenia, despite advanced disease stage/impaired liver function reserve, compared to those with non‐ALC in real‐world clinical settings. However, older age was strongly associated with sarcopenia, even in patients with ALC. There was no significant influence of heavy alcohol consumption on the development of sarcopenia.

Isolated tricuspid valve surgery–impact of etiology and clinical presentation on outcomes

To identify determinants of in-hospital and mid-term outcomes after isolated tricuspid valve surgery (ITVS) as little is known regarding the impact of tricuspid regurgitation (TR) mechanism and clinical presentation. Among 5661 consecutive adult patients who underwent a tricuspid valve surgery at 12 French tertiary centers in 2007-2017 collected from a mandatory administrative database, we identified 466 patients (8% of all tricuspid surgeries) who underwent an ITVS. Most patients presented with advanced disease (47% in NYHA III/IV, 57% with right-sided heart failure (HF) signs). TR was functional in 49% (22% with prior left-sided heart valve surgery and 27% isolated) and organic in 51% (infective endocarditis in 31% and other causes in 20%). In-hospital mortality and major complications rates were 10% and 31% respectively. Rates of survival and survival free of HF readmission were 75% and 62% at 5 years. Patients with functional TR incurred a worse in-hospital mortality than those with organic TR (14.4% vs. 6.3%, P = 0.004) but presentation was more severe. Independent determinants of outcomes were NYHA class III/IV (OR = 2.7[1.2–6.1], P = 0.01), moderate/severe right ventricular dysfunction (OR = 2.6[1.2–5.8], P = 0.02), lower prothrombin time (OR = 0.98[0.96–0.99], P = 0.008) and with borderline statistical significance, right-sided HF signs (OR = 2.4[0.9–6.5], P = 0.06) while TR mechanism was not (OR = 0.7[0.3–1.8], P = 0.88). ITVS was associated with high mortality and morbidity, both in-hospital and during follow-up, predicted by the severity of the presentation but not by TR mechanism. Our results suggest that TV intervention should be performed earlier in the course of the disease.

The learning curve for piggyback liver transplantation: identifying factors challenging surgery

BackgroundThis study aimed to quantify the learning curve of piggyback liver transplantation and to identify factors that impact the operative time and blood transfusion during the learning curve. MethodsA retrospective review was performed on consecutive cases of patients’ first piggyback liver transplantations that were performed by a single surgeon. The learning curve for the operative time was evaluated using the cumulative sum method. ResultsThere were 181, consecutive, first-time piggyback liver transplantations. The median operative time was 345 minutes (range: 180–745 minutes) with a median transfusion rate of 4 packed red blood cell units (range: 0–23 units). The cumulative sum learning curve identified 3 phases: an initial phase (1–70 piggyback liver transplantations), a plateau phase (71–101 piggyback liver transplantations), and a stable phase (102–181 piggyback liver transplantations). Over the 3 phases, there were significant decreases in the median duration of the surgery (388.8 vs 344.8 vs 326.9 minutes; P = .004, P = .0004, P ≤ .0001) and the number of red blood cell units transfused (6.00 vs 3.90 vs 3.71; P = .02, P = .79, P = .0006). Multivariable analysis identified that the following factors impacted the operative time: surgeon experience (P = .00006), previous upper abdominal surgery (P = .01), portocaval shunt fashioning (P = .0003), early portal section (P = .00001), multiple arterial graft reconstruction (P = .03), and the length of the retrohepatic inferior vena covered by segment 1 (P = .0006). Independent risk factors for increased blood loss were surgeon experience (P = .0001), previous upper abdominal surgery (P = .002), the retrohepatic inferior vena cava encirclement by segment 1 (P = .0001), severe portal hypertension (P = .01), early portal section (P = .001), and low prothrombin time (P = .00001). ConclusionEasily identifiable factors related to recipients (segment 1 morphology, previous upper abdominal surgery, severe portal hypertension) and to surgeon (operative experience, portocaval shunt fashioning, early portal section, and multiple arterial reconstructions) impact operative time and blood loss during the learning curve of piggyback liver transplantation. These factors can be used for grading the difficulties of liver transplantation to tailor the surgical strategy.

Predicting 90-day mortality at admission and 7 days post-admission among patients with hepatitis B virus-related acute-on-chronic liver failure.

To investigate the relationship between baseline characteristics and 90-day mortality in hepatitis B virus-related acute-on-chronic liver failure patients. The retrospective study was conducted at Fuling Centre Hospital, Chongqing, China, and comprised data from July 2015 to June 2018 of hepatitis B virus-related acute-on-chronic liver failure patients. Demographic characteristics and clinical features at admission and 7 days post-admission were noted. The data was then divided into two groups based on a patient's 90-day survival status, and their clinical and lab characteristics were compared using SPSS 16. Of the 120 patients screened, 100(83.3%) were included; 75(75%) males and 25(25%) females. The overall mean age was 50.04±14.61 years. There were 68(68%) in the surviving group and 32(32%) in the non-surviving group. Patients who had hyper-leukocytosis, hypoalbuminaemia, lower prothrombin time activity, ascites, hepatic encephalopathy, higher alanine aminotransferase levels and renal dysfunction at admission had poor prognoses (p<0.05). At 7 days post-admission, the non-surviving group had lower platelet count, higher aspartate aminotransferase level, lower bilirubin normalisation rate and higher total bile acid levels (p<0.05). Baseline organ failure severity was found to determine the outcome more strongly than the underlying cause.