Febrile infection -related epilepsy syndrome(FIRES)is a newly named entity of epileptic encepha-lopathy in recent years.As one of the hot spots studied by domestic and foreign scholars,the etiology and pathogenesis of FIRES,which has a poor treatment and outcome,are undefined.It manifests with refractory seizures and status epilep-ticus in previously healthy school -aged children after banal febrile illness.In addition,with the negative response to currently available treatments,there are a poor prognosis and a high mortality rate in FIRES.Survivors are typically left with refractory partial epilepsy and severe cognitive impairment.Through domestic and foreign literature,this article re-views the potential pathogenesis,clinical features,treatment and prognosis of the syndrome,and provides theoretical ba-sis and new ideas for clinical diagnosis and treatment.

Three kinds of fortified biscuits and bread were given as breakfast for 5 months to 106 preschool children 4-6 years old lodged in a kindergarten, with a control group taking common biscuits and bread without fortification. 6 g protein and 160 kcal were supplied by 50 g of fortifed foods in which 0.25 g lysine, 0.25 g lysine and 0.8 mg riboflavin, and cod liver oil and calcium in addition to lysine and riboflavin were fortified respectively. The results showed that a diet with intake of 2.1 g protein and 71 kcal/kg body weight fortified on the average by 228 mg lysine or 228 mg lysine and 1.1 mg riboflavin increased the monthly increment of body weights, heights, and urinary excretions of creatinine per day. There was also a tendency of improvement, in nitrogen retention. The urinary excretion of riboflavin in load test was increased in the group taking the second kind of fortified foods.

Pancreas of 53 aborted fetuses ranging from 5 to 41 weeks were fixed in Bouin's fluid and embedded in paraffin, and the sections were stained with Alcian bluephloxine and Grimelius silver technique. The A, B and D cell of islet of Langerhans were demonstrated with immunohistochemical PAP method also. 14 pancreas among them were fixed in 2% glutaraldehyde and embedded in Epon 812, and observed with TEM. Typical A, B and D islet cells were observed with LM at 12 weeks of fetal age. Undifferentiated, differentiated and degenerated cells were distinguished in the pancreatic islets at 14 to 41 weeks of fetal age. The secretory granules of the exoerine cells were observed after 14 weeks of fetal age. The functional differentiation of endocrine cells precede that of exocrine cells. The morphometrical measurement testified that the endocrine portion of fetal pancreas was obviously larger than that of adult. This study provides some morphological data for the pancreas transplantation in clinical medicine.

Objective To evaluate the pancreaticojejunostomy procedures selection strategy in pancreaticoduodenectomy.Methods The clinical data of 455 patients who received pancreaticoduodenectomy at the Xijing Hospital from June 2007 to June 2012 were retrospectively analyzed.For patients with pancreatic duct diameter≥4 mm,duct-to-mucosa pancreaticojejunostomy(DMPJ)was applied(DMPJ group,210 cases).For patients with pancreatic duct diameter ＜ 4 mm,modified Child pancreaticojejunostomy was applied to 140 patients(modified Child group)whose jejunal end was smaller than the pancreatic stump,and binding pancreaticojejunostomy was applied to 105 patients(binding group)whose jejunal end was bigger than or equal to the pancreatic stump.The clinical efficacy and incidence of postoperative complications were compared among the 3 groups.The count data and measurement data were analyzed by chi-square test and t test,respectively.Results The pancreatic duct diameter of the DMPJ group was(4.4 ± 0.7)mm,which was significantly bigger than(2.8 ± 0.6)mm of the modified Child group and(2.3 ± 0.7)mm of the binding group(t =2.25,2.48,P ＜ 0.05).The diameter of the pancreatic stump of the modified Child group was(36 ± 5)mm,which was significantly bigger than(21 ± 6)mm of the binding group(t =21.65,P ＜ 0.05).The overall incidence of pancreatic leakage was 8.4％(38/455).There were no significant differences in the incidences of pancreatic leakage,peritoneal bleeding,abdominal infection,digestive dysfunction rate and the mean duration of hospital stay among the 3 groups(x2 =0.53,0.88,1.63,5.34,F =2.53,P ＞ 0.05).Conclusion Pancreaticojejunostomy procedure selection strategy based on the diameters of pancreatic duct and pancreatic stump could obtain good clinical efficacy and is appropriate.

Objective To investigate the efficacy of rituximab-containing regimen in Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH). Methods A retrospective analysis involving 6 EBV-HLH patients who had received treatment with rituximab-containing regimen was performed. The patients who were diagnosed with lymphoma or primary HLH subsequently were not included in the analysis. Results All patients were males. The median age was 27.5 years (range 20-61 years). Two patients received rituximab-containing regimen as primary therapy, and got partial remission (PR) within 2 weeks after the first course of rituximab, but relapsed within 4 weeks. Four patients received rituximab-containing regimen as salvage therapy, but none achieved remission. The 6 patients died due to HLH and complications, such as infection and hemorrhage. Laboratory data including white blood cell count, haemoglobin concentration, platelet count ferritin, alanine transaminase, aspartate transaminase,total bilirubin, fibrinogen and EBV-DNA did not show statistical significance (all P>0.05). Conclusion The efficacy of rituximab as a treatment for EBV-HLH is not as good as that in the previous study, and a prospective clinical trial of rituximab-based monotherapy is needed to answer the question.

Objective To evaluate the changes in the expression of c-fos protein in the spinal cord in a rat model of oxycodone dependence or withdrawal response.Methods Thirty SPF adult male Sprague-Dawley rats,aged 6-8 weeks,weighing 180-220 g,were divided into 3 groups (n=10 each) using a random number table method:normal saline group (group NS),oxycodone dependence group (group OD),and oxycodone withdrawal group (group OW).In OD and OW groups,oxycodone was injected subcutaneously in back,5 days in total,with the dose of 2,3,4,5 and 6 mg/kg in turn,3 times a day (8:00/15:00/22:00).The equal volume of normal saline was given instead in group NS.The mechanical paw withdrawal threshold was measured at 3 days before administration and 30 min after the last administration every day.The oxycodone withdrawal was induced by intraperitoneal injection of naloxone 4 mg/kg at 8 h after the last administration of oxycodone on 5th day in group OW.The withdrawal response scores and range of weight changes were recorded within 15 min after giving naloxone or normal saline in NS and OW groups.Spinal cord tissues were collected at 1 h after the last administration on 5th day in group OD and at 1 h after giving normal saline or naloxone on 5th day in NS and OW groups for determination of the expression of c-fos protein by Western blot.Results Compared with group NS,the mechanical paw withdrawal threshold was significantly increased on 1 and 2 days after administration,and the expression of c-fos protein in the spinal cord was up-regulated in OD and OW groups,and withdrawal response scores were significantly increased,and the range of weight change was increased in group OW (P＜0.05).The expression of c-fos protein was significantly down-regulated in group OW as compared with group OD (P＜0.05).Conclusion Oxycodone dependence or withdrawal response may be related to the expression of c-fos protein in the spinal cord of rats,and the expression is up-regulated during oxycodone dependence,while down-regulated during oxycodone withdrawal.

OBJECTIVETo investigate the efficacy of liposomal doxorubicin together with etoposide and high dose methylprednisolone (DEP) as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis (HLH).

METHODSTotal 41 patients with refractory HLH were enrolled in this study. The efficacy of treatment with DEP regimen after 2 and 4 weeks were evaluated according to the United States Midwest Cooperative HLH Group.

RESULTSOf 41 refractory HLH patients, 28 were males and 13 females. The median age was 31(18-62) years old. The overall response rate (ORR) was 78.1%(32/41), including 12 patients (29.3%) achieved complete remission (CR) and 20 (48.8%) achieved partial remission (PR). The underlying disease of HLH were identified in 33 patients, including 1 case of primary HLH (CR), 20 cases of lymphoma associated HLH and 12 cases of EBV associated HLH. There were still 8 cases with unknown underlying disease. The 9 patients who had no response to DEP died within 2 to 4 weeks after salvage therapy. Twenty of the 32 patients who achieved PR or CR survived to undergo subsequent chemotherapy, allogenic hematopoietic stem cell transplantation (allo-HSCT) or splenectomy.

CONCLUSIONThe single-arm study suggested that DEP regimen appeared to be an effective salvage protocol for adult patients with refractory HLH.

Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; administration & dosage ; therapeutic use ; Cisplatin ; administration & dosage ; Etoposide ; administration & dosage ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphohistiocytosis, Hemophagocytic ; drug therapy ; Male ; Middle Aged ; Prednisone ; administration & dosage ; Remission Induction ; Salvage Therapy ; Young Adult

OBJECTIVETo analyze the clinical manifestations, laboratory data, therapy, and prognosis in patients with hemophagocytic lymphohistiocytosis (HLH).

METHODSA retrospective study was carried out in 192 adult patients with HLH between 2003 and 2013.

RESULTSOf the 192 cases, 70 cases were secondary to cancer and 64 cases secondary to infection. According to HLH-2004 criteria, the coincidence rate of indices were: fever (98.96%), high level of serum ferritin (94.27%), increased level of soluble interleukin- 2 receptor(sCD25) (94.79%), decreased or absent activity of NK cells (94.27%), cytopenias (80.73%), splenomegaly (80.21%), emophagocytosis in bone marrow, spleen or lymph nodes (74.48%), hypofibrinogenemia (50.52%), hypertriglyceridemia (37.50%). In addition, 94.27% of patients were presented with liver dysfunction, 96.35% with infections, and 75.52% with coagulopathy. Incidences of central nervous system symptoms and rash were 19.27% and 20.31%, respectively. Among cancer, infection and rheumatic group, there were statistically differences on white blood cells (WBC), platelet (PLT), sCD25, alanine aminotransferase, aspartate aminotransferase, total bilirubin and globulin(GLO) (P<0.05). The differences of WBC, PLT, albumin (ALB), GLO, brain natriuretic peptide, creatinine, urea nitrogen between survival group and death group had statistical significance.

CONCLUSIONThe secondary HLH occurs from various underlined diseases. Cancer, especially T- cell lymphoma, is the main cause, Secondly, it is EB virus infection. The diagnostic sensitive indicators are Persistent fever, higher level of serum ferritin, low or absent NK-cell activity, and increased sCD25 were the most valuable parameters for diagnosis. Cytopenias were not common in early phase of HLH secondary to rheumatic diseases. WBC, PLT, ALB, GLO could be used as the preliminary parameters for diagnosis. Cardiac insufficiency, renal insufficiency and coagulation dysfunction play important roles in prognosis.

Adult ; Bone Marrow ; Fever ; Humans ; Killer Cells, Natural ; Lymphohistiocytosis, Hemophagocytic ; Prognosis ; Retrospective Studies

OBJECTIVETo investigate the outcomes, survival status, and the prognostic factors of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) patients.

METHODSA retrospective study was carried out to analyze the clinical data of 61 EBV-HLH cases, from January 2008 to July 2014. Prognostic factors were analyzed through COX model (single factor and multiple factors).

RESULTSA total of 246 patients with HLH were diagnosed, among which 102 cases were with EBV infection (including 61 EBV-HLH, 36 lymphoma associated HLH, 5 primary HLH. Among the 61 cases, 40 were male and 21 were female, with a ration of 1.9:1. The median age was 28 years (range, 12-78). 1, 3, 6 and 12-month overall survival rates of 61 EBV-HLH were 65.6%, 47.5%, 32.4%, and 25.0%, respectively. The median follow-up time was 3 (0.5-28) months. 12 patients didn't use etoposide within 4 weeks after diagnosis, while HLH-94 protocol was used in 33 patients and HLH-2004 protocol was used in 16 patients. Response rates of theses three groups were 33.3%, 51.5%, and 43.8%, respectively (P=0.401). There was statistically difference between the group without etoposide and the HLH-94/ HLH-2004 group in the overall survival rate (P=0.033). Serum albumin level (P=0.033) and whether EBV could became negative (P=0.010) were independent predictors for EBV-HLH.

CONCLUSIONEBV-HLH patients have severe clinical feature and poor prognosis. Early application of immune chemotherapy based on etoposide can improve survival. Serum albumin level and whether EBV can become negative are independent prognostic factors for survival.

Adolescent ; Adult ; Aged ; Child ; Epstein-Barr Virus Infections ; Etoposide ; Female ; Herpesvirus 4, Human ; Humans ; Lymphohistiocytosis, Hemophagocytic ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Survival Rate ; Treatment Outcome ; Young Adult