Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy, there is no standard treatment and the prognosis is very poor. Affiliated Zhongshan Hospital of Dalian University report a case of 85-year-old BPDCN male patient treated with DVT regimen (decitabine combined with Venetoclax and thalidomide) and achieved complete remission. The patient with skin nodules and the pathology diagnosed BPDCN, the next generation sequencing of skin nodules showed mutations of IDH2 and ASXL1. DVT (decitabine combined with Venetoclax and thalidomide) has significant efficacy with rapid and deep remission for BPDCN, and the adverse effects is less, especially suitable for elderly patients who cannot tolerate intense chemotherapy.

Objective:To investigate the clinical features and family gene mutation results of the adult onset Gaucher disease patient.Methods:The diagnosis and treatment of a patient with Gaucher disease who was admitted to the Affiliated Zhongshan Hospital of Dalian University in March 2020 were retrospectively analyzed, and the literature was reviewed.Results:The patient (the proband) was a 34-year-old female, the anemia, thrombocytopenia and splenomegalia were found in 2005. After regular physical examination, the decrease of leukocyte, red blood cell and platelet was gradually aggravated, and the progressive enlargement of the spleen was aggravated. She visited the clinic on March 19, 2020 due to worsening of fatigue. The physical examination revealed hepatomegaly, splenomegaly; blood routine examination showed pancytopenia, and bone marrow morphology showed a large number of Gaucher cells. Peripheral blood β- glucocerebrosidase (GBA) of the proband was 1.8 nmol·mg -1·h -1 (reference value 10-25 nmol·mg -1·h -1), and the GBA of her father, mother, brother, and daughter was normal. The second-generation sequencing results showed that the proband had a mutation in the BCL2 gene at locus c.127G>A p.A43T, with a mutation frequency of 49.53%. GBA gene testing showed that the proband had a heterozygous mutation at c.1448T>C (a pathogenic mutation), heterozygous mutations at c.1026A>G and c.1038C>T (homozygous mutations), and a heterozygous mutation at c.1075G>T (mutation of unknown clinical significance). The proband's father had c.1448T>C heterozygous mutation, and he was a carrier; the proband's mother had c.1075G>T heterozygous mutation; the proband's brother had no gene mutation; the proband's daughter had c.1448T>C heterozygous mutation, and she was a carrier. The diagnosis was type Ⅰ Gaucher disease (non-neuropathic). The patient was proposed to participate in the clinical trial of imiglucerase, and during the follow-up, the blood routine was relatively stable, and the spleen remained progressively enlarged. Conclusions:Gaucher disease is rare, which is easy to be misdiagnosed and underdiagnosed. It is necessary to be alert to the possibility of Gaucher disease when there are patients with unexplained hemocytopenia, liver and spleen enlargement and multiple organs involvement, and enzyme and genetic tests should be performed as soon as possible.

Objective To construct and apply an intelligent follow-up information system for patients with cancer pain,providing references for improving the efficiency of hospital follow-up and promoting pain management of patients at home.Methods The intelligent discharge follow-up system for patients with cancer pain includes 2 platforms,namely a patient self-report platform and an administrator operation platform.The administrator operation platform consists of 5 modules,namely the workbench module,the follow-up plan module,the follow-up results module,the health education module and the data statistics module.In January 2022,the system was officially put into clinical application.The use of the system was analyzed,and patients'completion rate,medication compliance,incidence of moderate and severe pain and satisfaction with pain control were compared before(from January 2020 to December 2021)and after(from January 2022 to November 2023)the application of the system.Results At present,this system has been applied in 95 cancer-related wards of our hospital.From January 2022 to November 2023,the number of people who should be followed up was 4 248,and the number of people who actually completed the follow-up was 4 127;the rate of follow-up completion was 97.2%;the rate of timely completion of the follow-up was 94.9%;the rate of automatic follow-up by the system was 40.1%;the rate of patient abnormality report was 31.9%;the rate of timely treatment of patient abnormality report was 89.1%.After the application of the system,the completion rate of pain follow-up was increased,and the difference was statistically significant(P<0.001).After the application of the system,the medication compliance rate of patients with cancer pain increased from 86.9%to 91.0%;the incidence of moderate and severe pain decreased from 6.8%to 5.2%;the satisfaction with pain control increased from 81.0%to 83.5%(P<0.05).Conclusion The intelligent discharge follow-up system for patients with cancer pain can effectively improve the discharge follow-up efficiency and promote the management of patients with cancer pain at home.

Objective:To understand the degree of professional autonomy of nurses in Pediatric Nursing Alliance and the status of pediatric nursing practice environment, which providing guidance for the development of a series of specialized training in the alliance.Methods:Stratified random sampling method was used to conduct a questionnaire survey on nursing staff of different professional levels in Pediatric Nursing Alliance, which through the questionnaire star by using the questionnaire general information and training demand questionnaire, nurses practice professional autonomy scale, pediatric nursing staff structural empowerment questionnaire and nursing practice influencing factors questionnaire through the questionnaire star.Results:The total score for professional autonomy of nurses in the pediatric alliance was 192.66±18.63, the structural empowerment ( OR=1.137, 95% CI=1.084-1.194), lack of caring team ( OR=2.763, 95% CI=1.443-5.292) and performance evaluation ( OR=0.498, 95% CI= 0.274-0.908), specialized education and professional experience ( OR=0.548, 95% CI= 0.334-0.871) were affecting the clinical nursing practice. Conclusion:The degree of professional autonomy of nurses in the Pediatric Nursing Alliance is in the middle and high level. Key factors affecting nursing practice including insufficient structural empowerment, lack of opportunities to continue learning, lack of nursing teams, lack of effectiveness evaluation and the lack of specialized education and work experience, which guiding the pediatric nursing alliance to continuously deepen the connotation of pediatric nursing professional and innovative team collaboration new model, utilize the advantages of resources to actively cultivate specialized nursing research personnel, carry out multi-disciplinary and cross-disciplinary cooperation, improve the nursing quality evaluation index system, so as to enhance the professional nursing service capacity and value.

Objective:To explore the value of mammography-based radiomics for preoperative prediction of axillary lymph node metastasis in breast carcinoma.Methods:The clinical and X-ray data of female patients with pathologically confirmed breast cancer in Henan People′s Hospital from June 2013 to July 2017 were analyzed retrospectively. A total of 214 patients, aged 30-85 (53±11) years, were randomly divided into training set ( n=153) and verification set ( n=61) according to the ratio of 3∶1. According to pathological findings of the axillary lymph node metastasis, 99 cases were divided into positive group and 115 cases into negative group. The lesions were segmented and extracted in X-ray images of mediolateral oblique (MLO) and cranial caudal (CC). Three, nine and seven axillary lymph node metastasis related histologic features were selected from the high dimensional features of CC, MLO and CC combined MLO images by lasso regression model. According to the characteristics of imaging and clinical characteristics, the prediction model was constructed. The prediction ability of the model was verified by 10% cross validation. Results:The lymph node in positive group was larger than negative groups, the difference was statistically significant ( t=2.611, P<0.05). In the validation set, the area under curve (AUC) values of CC, MLO, CC combined with MLO images, clinical features and clinical features combined with CC and MLO images were 0.680, 0.723, 0.740, 0.558 and 0.714, respectively. Among them, CC combined with MLO images had the highest prediction efficiency, and AUC values were higher than CC alone, MLO images and CC combined with MLO images. Conclusions:Quantitative radiomics features of breast tumor extracted from digital mammograms are helpful for preoperatively predicting axillary lymph node metastasis. Future larger studies are needed to further evaluate these findings.

Objective:To investigate the imaging and pathology features of rosette-forming glioneuronal tumor (RGNT).Methods:From January 2010 to September 2019, 7 patients with pathologically conformed RGNT in our hospital were chosen, the imaging findings were analyzed retrospectively and compared with corresponding pathological features.Results:Six patients showed single lesion: 4 located in the vermis and its surrounding areas, in which 2 involved the pineal region and the tectum; the other 2 lesions located in the fourth ventricle. One patient had multiple lesions, which located in the third ventricle and the lateral ventricle respectively. Cystic-solid masses mainly with cystic changes were noted in these 7 patients, lesions in the vermis of cerebellum and its surrounding areas were polycystic while lesions in the ventricles were single cystic. The cystic areas of masses showed low density on plain CT scan and slightly long T 1 and long T 2 signal on MR imaging. The imaging findings were compared with the pathological findings: the density/signal changes were corresponded to the cystoid changes, hemorrhage and calcification, as seen in the postoperative specimens; the signal changes of the cystic areas were corresponded to the mucus matrix components and old hemorrhage; the characteristics of tumor enhancement were closely related to the distribution of solid components and degrees of blood supply. Conclusion:The imaging features of RGNT may partly reflect its pathological manifestations; combined with the specific location, imaging examinations may help make a correct preoperative diagnosis.

Objective To explore the value of various diffusion parameters obtained from monoexponential, biexponential and stretched exponential diffusion-weighted imaging models in assessing hepatic fibrosis in chronic hepatitis B(CHB). Methods A total of 52 patients who were diagnosed hepatitis B by the markers of hepatitis and were confirmed by liver biopsy pathology were prospectively recruited between June 2014 and May 2016 in People's Hospital of Henan Province. Concomitantly, 30 healthy volunteers who had no history of hepatitis B and liver dysfunction were enrolled in the control group. All patients underwent multi-b values DWI on a 3.0 T MRI unit. ADC was calculated by using the monoexponential model. True diffusion coefficient(Dt),pseudo diffusion coefficient(Dp)and fraction of perfusion(f)were calculated by using the biexponential model.Distributed diffusion coefficient(DDC)and water molecular diffusion heterogeneity index(α)were calculated by using the stretched exponential model. Liver biopsy specimens were staged according to the degree of hepatic fibrosis (S0 to S4). The Kruskal-Wallis test was employed for the comparison of each parameter among the control group and the fibrosis stage groups. The Mann-Whitney U test was adopted to compare each parameter between fibrosis stage≤S1and≥S2,between≤S2 and≥S3.Spearman rank correlation coefficients were obtained to assess the correlation of the parameters with the fibrosis stages.ROC analysis was used to evaluate the performance of various parameters in predicting stage≥S2 and≥S3.Results The hepatic fibrosis stage distributions were as follows:1 cases with S0,9 cases with S1,22 cases with S2,11 cases with S3,9 cases with S4.ADC,Dt,f and DDC values all showed significant difference among the control group and groups S1,S2,S3,S4(all P<0.05), while Dp and α values showed no significant difference(P>0.05). Dt, DDC and ADC showed a moderate negative correlation with the fibrosis stage(r=-0.630,-0.603 and-0.464,respectively,all P<0.01),and f showed a mild negative correlation with the fibrosis stage(r=-0.379,P<0.05),while Dp and α values showed no correlation with the fibrosis stages(all P>0.05).The ADC, Dt, f and DDC values all showed significant difference between stage≥S2 and≤S1,between stage≥S3 and≤S2(all P<0.05),and the parameter values of the former were greater than those of the latter. While Dp and α values showed no significant difference among both groups(all P>0.05).The AUCs of ADC,Dt,f and DDC values for detecting fibrosis stage ≥S2 were 0.738,0.835,0.740 and 0.831, and the AUCs of ADC, Dt, f and DDC values for detecting fibrosis stage≥S3 were 0.716, 0.811, 0.672 and 0.798. Conclusion The Dt derived from biexponential and DDC derived from stretched exponential DWI could be useful for the staging of hepatic fibrosis in CHB.

Objective To investigate the damage of retinal ganglion cell (RGC) axons in retinitis pigmentosa (RP) quantitatively by using reduced-filed-of-view DTI (rfov-DTI) together with optical coherence tomography(OCT).Methods Thirty four patients(68 eyes,Patient group)and 33 healthy controls (66 eyes, Control group) were enrolled in this study. Measures of rfov-DTI and OCT of both eyes in all subjects were performed by 3.0 MRI. The difference of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (λ//) and radial diffusivity (λ⊥) were compared by two independent sample t test. The peripapillary retinal nerve fiber layer (RNFL) thickness in temporal and lateral nose, which data don't conform to normal distribution, were calculated by average and four quadrants and compared by Mann-Whitney U test,the average RNFL thickness and RNFL thickness in low quadrant and upper quadrant were compared by two independent sample t test.Spearman rank were performed to determine the correlation between DTI parameters of optic nerves (ONs) and RNFL thickness. Results The value of FA, MD, λ//, λ⊥, average, temporalside, nasal side, low quadrant and upper quadrant RNFL thickness in patient group were 0.445±0.078,(1.330±0.236)×10-3mm2/s,(5.502±0.263)×10-3mm2/s,(4.494±0.238)×10-3mm2/s,(104.7± 25.4)μm,(104.0±31.0)μm,(65.0±25.0)μm,(122.0±33.3)μm,and(117.4±37.7)μm respectively and the data in control group were 0.581±0.043,(1.079±0.146)×10-3mm2/s,(5.349±0.214)×10-3mm2/s,(4.195±0.126)× 10-3mm2/s, (101.2 ± 10.6)μm, (70.0 ± 10.8)μm, (81.3 ± 13.4)μm, (127.3 ± 12.5)μm, (126.3 ± 11.5)μm respectively.Compared with the control group,the value of MD,λ//,λ⊥were significantly higher but FA was significantly lower (t=-12.388, 7.394, 3.695, 9.062;all P<0.01). The temporal RNFL was significantly thicker while the nasal RNFL was much thinner than that in control group respectively(Z=-8.384,-4.518;all P<0.01);the average, low quadrant and upper quadrant RNFL thickness showed no significant differences compared with the control group(t=-1.217,-1.843, 1.049;P=0.227, 0.069, 0.297).Especially,there was a weak correlation between the temporal RNFL thickness and FA(r=0.268,P=0.029),however,no correlation of nasal RNFL thickness with mean FA was found(r=0.164,P=0.186). Conclusions rFOV-DTI together with OCT can provide information about the pathology of RGC axon disease in patients with RP.there is a weak correlation between the retinal nerve fiber layer and the pathological changes of optic nerve in the retinal ganglion cells,but the performance were not coincident.

Objective To investigate the clinical symptoms and mental state of patients with irritable bowel syndrome with diarrhea (IBS-D ) , and to analyze the characteristics of psychological disorders in patients with IBS-D and their impacts on intestinal symptoms .Methods From July 2009 to June 2012 ,patients met Rome Ⅲ criteria of IBS-D were consecutively enrolled at Peking Union Medical College Hospital .The symptoms of IBS were investigated by IBS symptoms questionnaire and mental state were evaluated by Hamilton anxiety scale (HAMA ) and Hamilton depression scale (HAMD ) . The differences in intestinal symptoms between patients with comorbid psychological disorders and without psychological disorders were compared .And the correlation between the scores of HAMA ,HAMD and intestinal symptoms were analyzed . Two independent sample t-tests ,chi square test and Fisher exact probability were performed for statistical analysis .Spearman rank correlation was used for correlation analysis .Results A total of 231 patients with IBS-D were enrolled .There were 133 males and 98 females with an age of (42 .8 ± 11 .1) years old and a disease course of (4 .5 years (8 .0 years)) .The HAMA and HAMD scores were 17 .00 ± 7 .12 and 14 .05 ± 6 .00 ,respectively ,and 72 .29％ (167/231) patients had comorbid psychological disorders ,32 .90％ (76/231 ) patients had moderate to severe anxiety and/or depression ,mainly had anxiety .The proportion of patients with ordinary abdominal pain or discomfort and the proportion of moderate to severe abdominal pain or discomfort in patients with psychological disorders were higher than those of patients without psychological disorders (53 .29％ , 89/167 vs . 34 .37％ , 22/64;49 .44％ ,44/89 vs .18 .18％ ,4/22) ,and the differences were statistically significant (χ2=6 .634 and 7 .002 , P=0 .010 and 0 .009) .In patients with comorbid psychological disorders ,more patients had frequent onset of abdominal pain or discomfort ,less achieved completely improvement after defecation , and often accompanied with defecation related symptoms .The HAMD score was positively correlated with the onset frequency of IBS (r=0 .172 ,P=0 .009) ,and the HAMA score was positively correlated with the degree of abdominal pain or discomfort before defecation (r=0 .134 , P= 0 .042) .The HAMA and HAMD scores were negatively correlated with the improvement degree of abdominal pain or discomfort after defecation (r= -0 .215 , P=0 .001 ;r= -0 .251 , P<0 .01) ,and were positively correlated with waiting time for symptoms improvement (r=0 .175 , P=0 .008;r=0 .219 , P= 0 .001) .Conclusion Most IBS-D patients have comorbid psychological disorders , anxiety and/or depression greatly impact the intestinal symptoms of patients with IBS .

Objective To explore the value of mammogram texture analysis in prediction of metastasis of axillary lymph nodes for breast carcinoma.Methods Mammograms and clinical data of 171 patients with breast carcinoma confirmed by pathology were retrospectively analyzed,and all patients underwent axillary lymph node dissection (ALND).Then the patients were divided into axillary lymph node metastasis group and non-metastasis group according to the result of ALND.The texture features of these lesions were statistically analyzed,including gray-level histogram texture parameters (mean value,standard deviation,skewness,kurtosis and variance) and gray-level co-occurrence matrix texture parameters (energy,entropy,correlation,inertia,inverse difference moment and contrast).Results In all of 171 breast cancer patients,96patients had axillary lymph node metastasis,while 75 patients had no metastasis.Mammograms showed negative axillary lymph nodes in 119 patients and positive axillary lymph nodes in 52 patients,and the sensitivity and specificity of mammograms in the diagnosis of positive axillary lymph nodes was 48.96％ (47/96) and 93.33％ (70/75),respectively.Mammogram texture analysis showed the values of energy,entropy,inverse difference moment and correlation in axillary lymph node metastasis group were higher than those in non-metastasis group,while the values of inertia and contrast in the axillary lymph node metastasis group were lower than those in non-metastasis group (all P＜0.05).The rest texture parameters had no significant differences between two groups (all P＞0.05).Area under curve (AUC) for texture parameters of energy,entropy,inertia,inverse difference moment,correlation and contrast was 0.610,0.610,0.374,0.599,0.612 and 0.421 (all P＜0.05),respectively.AUC of mammography,mammogram texture features,and the combination of mammography and texture features was 0.711,0.676 and 0.787 (all P＜0.05),respectively.The sensitivity and specificity of mammogram texture features,the combination of mammography and texture features in diagnosis of axillary lymph nodes metastasis was 62.5％ and 64.6％,66.7％ and 82.7％,respectively.Conclusion Mammogram texture parameters are helpful for predicting axillary lymph node metastasis,and the combination of mammography and texture features can improve diagnostic efficiency of axillary lymph node metastasis.