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.

Compressed sensing (CS) is a novel theoretical framework for information acquisition and processing. Taking advantages of the sparsity or compressibility of the signals inherent in the real world, compressed sensing can collect compressed data at the sampling rate much lower than that needed in Shannon's theorem based on random measurement matrix. This technique is used in medical imaging to accelerate MRI's scanning speed, reduce radiation dosage and alleviate patients' suffering. The whole process of the proposed algorithm was as follows: firstly, the wavelet transform was applied to achieve sparse representation of medical images and reserve certain parts with maximal coefficients; secondly, the reconstruction based on CS theory were achieved according to the improved optimized orthogonal matching pursuit (OOMP) algorithm;finally, maximum intensity projection algorithm was used to achieve three-dimensional volume reconstruction. The experimental results demonstrated that our proposed two-dimensional reconstruction method was accurate and effective, which was verified qualitatively by the local detail magnification of images and quantitatively by peak signal-to-noise ratio and sectional comparison. Therefore, the three-dimensional reconstruction can be rather helpful in clinic diagnosis and treatment.

Objective:To investigate the effectiveness of multimodal magnetic resonance imaging (MRI)-based radiomics models in the auxiliary diagnosis of neonatal cerebral white matter lesion (WML).Methods:Clinical and MRI imaging data were collected from 91 children diagnosed with WML in Dalian Women and Children′s Medical Center from April 2018 to December 2021, including 58 cases in the good prognosis group and 33 cases in the poor prognosis group. The region of interest (ROI) was drawn for the lesion of each sequence image (T 1WI, T 2WI, DWI and SWI). Three models, T 1WI+T 2WI (model 1), T 1WI + T 2WI + DWI (model 2) and T 1WI + T 2WI + SWI (model 3), were created. Resample of the ROI, pre-processing of images and extraction of features were performed by using PyRadiomics, and the extracted features were standardized on the Dr.Wise research platform. A logistic regression classifier was used to create the radiomics model and 5-fold cross-validation was carried out 10 times. Key features were screened, and the receiver operating characteristic (ROC) curve was drawn to evaluate the predictive ability of the models. And DeLong was used to compare the effectiveness of different models. Results:The AUC values of model 2 (0.95) and model 3 (0.93) in the validation set were higher than that of model 1 (0.90). The accuracy, sensitivity and specificity of model 1, model 2, and model 3 were (86%, 89% and 87%), (79%, 88% and 88%) and (90%, 90% and 86%), respectively. The differences between model 1 and model 2 by DeLong′s test was statistically significant ( P<0.05), whereas there was no statistically significant differences between model 3 and model 1 or between model 3 and model 2 ( P>0.05). Conclusions:Multimodal MRI-based radiomics model was proved to be an effective tool in the auxiliary diagnosis of neonatal WML.

Objective:To explore the predictive value of N-terminal-pro-B-type brain natriaretic peptide (NT-pro BNP) and B-type brain natriaretic peptide (BNP) of symptomatic patent ductus arteriosus (sPDA) in premature infants.Methods:Sixty premature infants were recruited. The gestational age was 26-32 weeks and the birth weight was less than 1 500 g. According to the echocardiography measured on the 3rd day, the infants were divided into PDA group (40 cases) and control group (20 cases). PDA group was divided into sPDA group (20 cases) and asPDA group (20 cases). The sPDA group was divided into treatment group (10 cases) and non-treatment group (10 cases). The asPDA group was further divided into self-closed group (10 cases) and non- self-closed group (10 cases). NT-pro BNP and BNP were examined on the 3rd and 7th day.Results:The level of NT-pro BNP among sPDA group, asPDA group and control group had significant difference on the 3rd and 7th day ( P<0.05). On the 7th day, the level of NT-pro BNP between treatment group and non-treatment group had significant difference [4 082(111 - 7 566) ng/L vs. 9 643 (2 362 - 25 000) ng/L, P<0.05] between self-closed group and non- self-closed group [603 (27 - 1 698) ng/L vs. 4 595 (896 - 9 873) ng/L, P<0.05]. The level of NT-pro BNP on the 3rd day was positively correlated with of ductus arteriosus diameter, ratio of left atrium diameter and aortic root diameter, ratio of ductus arteriosus diameter and left pulmonary artery diameter ( r=0.358 - 0.455, P<0.05). The area under the curve drawn by NT-pro BNP on the 3rd day was 0.783. Conclusions:The level of NT-pro BNP on the 3rd day can be used as a predictor of sPDA.

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