Hemophagocytic lymphohistiocytosis complicated by polyserositis: A case report.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare group of disorders of immune dysregulation characterized by clinical symptoms of severe inflammation. There are basically two types of clinical scenarios: Familial HLH and sporadic HLH. It is thought that the syndrome is implicated in the development of infections, malignancies, and autoimmune diseases. HLH, whether primary or secondary, is characterized by activated macrophages in hematopoietic organs, hepatosplenomegaly, cytopenia, and fever; however, HLH complicated with polyserositis (PS) has never been reported. CASE SUMMARY: We present a case of fever in a 46-year-old previously healthy Chinese woman complicated by pericardial, pleural, and abdomen effusions. She had no contact with sick individuals, recent travel, illicit drug use, or new sexual contacts. She did not consume alcohol or tobacco and lacked a family history of other diseases. Antibiotics were prescribed for suspected infection, and acute liver injury subsequently occurred. Contrast-enhanced computed tomography showed mild pericardial effusion, pleural effusion, hepatosplenomegaly, and a large amount of ascites. A full blood count revealed leukopenia and thrombocytopenia. Increased ferritin and triglyceride levels were observed. The test for Epstein-Barr (EB) virus DNA was positive. This suggests that EB virus replication and EB virus infection existed. Additional studies showed hemophagocytosis in bone marrow biopsy specimens. The patient's condition progressed rapidly. After providing symptomatic support treatment, eliminating immune stimuli, and administering comprehensive cyclosporine and dexamethasone treatment, the patient's condition continued to progress, and the patient's family members decided to stop treatment; the patient subsequently died. CONCLUSION: This case shows the significance of considering HLH as part of the evaluation of unexplained fever and PS of unknown origin.

Binding characteristics of plasma protein in active parts of Daidai lipid-lowering flavonoid extract / 中国中药杂志

To study the binding capacity of active ingredients of Daidai lipid-lowering flavonoid extract and plasma protein,investigate the ways to improve the traditional formula for calculating protein binding rates based on ultrafiltration,and increase the stability and reliability of the experimental results. UPLC-MS/MS was used to establish a quantitative analysis method for simultaneous determination of active ingredients( neohesperidin and narngin) in ultrafiltrate. The protein binding rates were calculated by the traditional ultrafiltration formula. The correction factors( F) were introduced later,and the binding rates calculated with the correction factors were compared with those without the correction factors. The binding capacity of the extract and plasma protein was evaluated. The quantitative analysis method established by UPLC-MS/MS had a good specificity. The standard curve and linear range,method accuracy,precision and lower limit of quantitation all met the requirements. The method met the requirement for quantitative detection of the active ingredients in ultrafiltrate after the rat plasma was filtrated in the ultrafiltration tube. Under the experimental conditions,the binding rates of both active ingredients( neohesperidin and narngin) were higher than 90%. The active ingredients and rat plasma protein were bound in a concentration-dependent manner,with statistically significant differences( P<0. 01). There was no statistically significant difference between the protein binding abilities of the two active ingredients with rat plasma protein. Therefore,the active ingredients of Daidai lipid-lowering flavonoid extract had a relatively strong binding strength with rat plasma protein,and they were bound in a concentration-dependent manner. Additionally,when calculating protein binding rates by the traditional ultrafiltration formula,the correction factors could be introduced to effectively reflect the errors of multiple ingredient groups in traditional Chinese medicine extracts.This correction method could provide a reference thinking and practical reference for the improvement of the determination method of the traditional Chinese medicine plasma protein binding ability based on ultrafiltration.

Metabolic characteristics of active parts of lipid-lowering flavonoid extract of Daidai in liver and intestinal microsomes of rats / 中国中药杂志

The paper studies and compares the metabolic difference of active ingredients of lipid-lowering flavonoid extract of Daidai in rat livers and intestinal microsomes,in order to explore the phase Ⅰ metabolism characteristics of active ingredients in livers and intestines. UPLC-MS/MS was used to establish a quantitative analysis method for active ingredients,neohesperidin and narngin,in a phase Ⅰ metabolism incubation system of liver and intestinal microsomes. Differential centrifugation was used to make liver and intestinal microsomes of rats. A phase Ⅰ metabolism incubation system was established,and the concentrations of the residual at different incubation time points were analyzed. Graphs were plotted to calculate the metabolic elimination half-life of the main active parts,with the natural logarithm residual percentage values ln( X) at different time points as the y axis,and time t as the x axis. The metabolism characteristics of the active ingredients were compared. The established UPLC-MS/MS quantitative analysis method has a good specialization,standard curve and linear range,accuracy and precision,with a satisfactory lower quantitative limit. The method allows quantitative detection of the active ingredients in a phase Ⅰ metabolism incubation system of liver and intestinal microsomes of rats. In the rats liver microsomes incubation system,the metabolic elimination half-life of neohesperidin and narngin were( 2. 20 ± 0. 28) h and( 1. 97±0. 28) h respectively. The elimination half-life of neohesperidin was larger than that of narngin,but with no statistically significant difference. In the rats intestinal microsomes incubation system,the metabolic elimination half-lives of neohesperidin and narngin were( 3. 68±0. 54) h and( 2. 26±0. 13) h respectively. The elimination half-life of neohesperidin was larger than that of narngin,with statistically significant differences( P<0. 05). The elimination half-lives of the active ingredients in liver microsomes were smaller than those in intestinal microsomes. The experiment results showed that the active ingredients of lipid-lowering flavonoid extract of Daidai had different elimination half-lives in phase Ⅰ rats liver and intestinal microsomes incubation system. This implied that they had different metabolic characteristics in rats liver and intestine,and liver may be the main metabolism site of the active ingredients. The phaseⅠ metabolism of narngin was stronger than that of neohesperidin. The differences between their metabolic characteristics may be related to the binding sites of B-ring hydroxyl in flavonoid glycosides and the number of methoxyl group. The results provided an important experimental basis for further development and clinical application of lipid-lowering flavonoid extract preparation of Daidai.

[Non-invasive assessment of portal hypertension in patients with liver cirrhosis using FibroScan transient elastography].

OBJECTIVE: To investigate the clinical value of FibroScan transient elastography for assessing portal hypertension in liver cirrhosis patients by determining the relationship between the liver or spleen stiffness measurement with the imaging parameters of esophageal varices, portal vein width, spleen volume, and splenic vein width. METHODS: A total of 259 patients with liver cirrhosis underwent FibroScan measurement, ultrasound, computed tomography and routine blood analyses. One-hundred-and-one of those patients also underwent endoscopy to diagnose esophageal varices. Receiver operating characteristic (ROC) curves were generated and the areas under the curves (AUCs) were calculated to assess the accuracy of the FibroScan liver and spleen stiffness measurements to predict esophageal varices. Pearson's correlation analysis was used to assess the relationship between clinical features. RESULTS: The median liver and spleen stiffness of the cirrhotic patients were 24.27 kPa and 44.64 kPa, respectively. Liver and spleen stiffness increased in conjunction with increases in Child-Pugh score. Liver stiffness was positively correlated with spleen stiffness (P less than 0.05). Liver and spleen stiffness were positively correlated with esophageal varices, portal vein width, spleen thickness, spleen volume, and splenic vein width. The correlation of spleen stiffness was higher than that of liver stiffness. Spleen stiffness was also negatively correlated with white blood cell count and platelet count. Liver and spleen stiffness also increased in conjunction with increased severity of esophageal varices. The AUC of spleen stiffness was higher than that of liver stiffness for predicting esophageal varices (0.804 vs. 0.737). The optimal cut-off level of spleen stiffness was 44.5 kPa (sensitivity: 88%; specificity: 68%). The estimated prevalence of esophageal varices was 97.87% and the optimized cut-off level of liver stiffness was 18.0 kPa. CONCLUSION: FibroScan appears to be a clinically valuable non-invasive method to assess portal hypertension in cirrhotic patients. Both liver and spleen stiffness measurements correlated with portal hypertension but the spleen stiffness measurement may be of higher clinical value.

[Analysis of prognostic factors of severe jaundice patients using HB-H-6 resin plasma perfusion].

OBJECTIVE: To investigate the influencing factors of efficacy of plasma perfusion in patients with severe jaundice. METHODS: The clinical data of 78 patients with severe jaundice due to different causes receiving HB-H-6 resin plasma perfusion therapy admitted to Tianjin Third Central Hospital from October 2006 to July 2010 were retrospectively analyzed. Patients were divided into improved group (n = 51) and ineffective group (n = 27) according to outcomes. The effecting factors of prognosis, including age, sex, hospital stay days, number of perfusion therapy received, Child-Pugh scores before perfusion, total bilirubin (TBil) levels before perfusion, and mean TBil rebound rate were studied by univariate and multivariate logistic regression analysis. RESULTS: All 78 patients received (3.31 ± 1.36) times of HB-H-6 resin plasma perfusion treatment. Child-Pugh score before perfusion, TBil (µmol/L) before perfusion and mean TBil rebound rate in improved group were significantly lower than those in ineffective group [Child-Pugh score before perfusion: 8.06 ± 1.01 vs. 9.44 ± 1.19; TBil before perfusion: 384.29 ± 170.41 vs. 504.93 ± 206.88; mean TBil rebound rate: -(7.35 ± 20.76)% vs. (37.32 ± 23.22)%]. They were also significantly different in gender between two groups (improved group: 30 males, 21 females; ineffective group: 24 males, 3 females, P < 0.05 or P < 0.01). Gender and mean TBil bounce rate were defined as independent significant factors influencing the clinical results by multivariate logistic regression analysis. Regression coefficient ß were 5.35 and -2.82 for gender and mean TBil bounce rate respectively [χ (2) = 64.42, P = 0.000]. Receive operating characteristic curve (ROC curve) analysis showed that the area under the curve (AUC) was 0.90 (0.82, 0.97), and mean TBil bounce rate higher than 29.5% indicated poor prognosis. No obvious side effects were observed after plasma perfusion. CONCLUSIONS: Gender and mean TBil bounce rate were independent risk factors in treatment of severe jaundice with HB-H-6 resin plasma perfusion. Mean TBil bounce rate higher than 29.5% indicated a poor prognosis.

[An experimental study of plasma-perfusion with a novel aminated chitosan on liver failure in a canine model].

OBJECTIVE: To study the efficacy and safety of plasma-perfusion with a novel aminated chitosan on liver failure in a canine model. METHODS: A canine model of liver failure was established. Plasma-perfusion with a novel aminated chitosan was performed on those dogs. Blood pressure and body temperature during plasma-perfusion were monitored. Total plasma bilirubin, direct bilirubin and indirect bilirubin at the entrance and exit of a column before and after plasma-perfusion and at the time of 15, 30, 60, and 120 min during plasma-perfusion were examined. Blood alanine aminotransferase, aspartate aminotransferase, ammonia, plasma prothrombin time, electrolytes and other blood parameters were examined before and after the plasma-perfusion. RESULTS: After the plasma-perfusion, total bilirubin decreased from 177.4+/-18.1 to 46.1+/-3.7 (P less than 0.05), direct bilirubin decreased from 124.2+/-10.3 to 30.5+/-1.7 (P less than 0.05), indirect bilirubin decreased from 53.2+/-2.8 to 15.6+/-2.0 (P less than 0.05). Compared at the entrance of the column, there were significant decreases in the levels of total bilirubin, direct bilirubin and indirect bilirubin of plasma at the exit of the column at the times of 15 and 30 min during plasma-perfusion (P less than 0.05); there were no further significant decreases at 60 and 120 min (P more than 0.05). Compared with pre-plasma-perfusion, there were significant decreases in the levels of blood alanine aminotransferase, aspartate aminotransferase, and ammonia (P less than 0.05); the plasma prothrombin time was significantly increased (P less than 0.05), the electrolytes, hematocrit level, platelet count, and white cell count were not affected significantly by the perfusion (P more than 0.05); blood pressure and body temperature were not affected significantly during the plasma-perfusion. CONCLUSION: Plasma-perfusion with a novel aminated chitosan resin is an effective and safe method for treating liver failure in this canine model.