Efficacy of Omalizumab in the treatment of moderate-to-severe allergic asthma with allergic comorbidities in children / 中华实用儿科临床杂志

Objective:To investigate the clinical efficacy of Omalizumab in the treatment of moderate-to-severe allergic asthma with allergic comorbidities in children.Methods:The clinical data of 50 children with moderate-to-severe allergic asthma and allergic comorbidities, who were treated with Omalizumab and completed 12-month follow-up in the Department of Pediatrics of Peking University First Hospital from July 2018 to March 2022, were retrospectively analyzed.A comparison was performed on the scale scores of childhood allergic asthma and allergic comorbidities including allergic rhinitis (AR) and chronic spontaneous urticaria (CSU), pulmonary function test indices and fractional exhaled nitric oxide (FeNO) concentration before and after treating with Omalizumab.The data were compared by ANOVA, paired t-test, chi- square test and rank sum test. Results:(1)Improvement of clinical symptoms: after 12 months of Omalizumab treatment, the childhood asthma control test score of 42 children aged ≤11 years increased from (20.98±5.03) points to (26.95±0.22) points ( F=18.189, P<0.001). The asthma control questionnaire 7 score of 50 children decreased from (0.89±0.11) points to (0.10±0.02) points ( F=5.662, P=0.006). The score of visual analogue scale of 47 children with AR decreased from (11.00±1.65) points to (0.2±0.14) points ( F=14.901, P<0.001), and the urticaria control test score of 13 children with CSU decreased from (4.82±0.88) points to (1.87±0.61) points ( F=4.329, P=0.018). (2)Improvement of quality of life: compared with those before treatment, the pediatric asthma quality of life questionnaire score in 50 children increased from (124.50±32.13) points to (159.40±6.21) points ( F=12.052, P<0.001), and global evaluation of asthma treatment effectiveness decreased from (2.23±0.70) points to (1.07±0.26) points ( F=68.865, P<0.001) after Omalizumab treatment for 12 months.(3)Improvement of pulmonary function results: after 12 months of Omalizumab treatment, the number of children with forced expiratory volume in one second/forced vital capacity< 80% decreased from 13 cases (26%) to 1 case (2%), and the values increased from (91.39±12.88)% to (96.96±8.54)%( χ2=11.960; t=2.486, all P<0.05). The peak expiratory flow of predicted value increased from (86.73±16.05)% to (94.01±13.11)% ( t=2.445, P<0.05). The number of children with two indicators among the forced expiratory flow at 50% of forced vital capacity exhaled, forced expiratory flow at 75% of forced vital capacity exhaled and maximal mid-expiratory flow lower than 65% decreased from 31 cases (62%) to 7 cases (14%) ( χ2=24.450, P<0.001). There was no significant difference in FeNO concentration before and after treatment ( P>0.05). Safety of Omalizumab: no obvious adverse reactions were found during treatment and follow-up. Conclusions:Omalizumab can significantly improve the clinical symptoms, small airway function and quality of life of children with allergic asthma and concomitant AR or CSU.It is a potential targeted drug for treating a variety of allergic diseases in children.

Case series study on efficacy and safety of Omalizumab in the treatment of moderate-to-severe allergic asthma in children / 中华实用儿科临床杂志

Objective:To explore the clinical efficacy and safety of the new targeted anti-immunoglobulin E (IgE) drug Omalizumab in the treatment of children with moderate and severe allergic bronchial asthma in China.Methods:The clinical data of children with moderate and severe allergic asthma who were treated in the Department of Pediatrics of Peking University First Hospital from July 2018 to January 2020 and treated with Omalizumab were retrospectively analyzed.A comparison was performed on the changes of questionnaires, including childhood asthma control test (C-ACT), pediatric asthma quality of life questionnaire (PAQLQ), mini-asthma quality of life questionnaire (Mini-AQLQ) and global evaluation of asthma treatment effectiveness (GETE), pulmonary function test, including forced expiratory volume in the first second (FEV 1), percentage to predicted value (FEV 1% pred) and small airway function, fractional concentration of exhaled nitric oxide (FeNO) and so on, before and after treating with Omalizumab. Results:A total of 15 pediatric patients completed 16 weeks of treatment follow-up.After 16 weeks of Omazumab treatment, the score of C-ACT increased from (16.001.66) scores to (25.38±0.64) scores ( F=11.969, P<0.001), PAQLQ increased from (118.08 ± 23.78) scores to (141.00 ± 11.91) scores ( F=9.289, P=0.001), Mini-AQLQ increased from (78.93±7.43) scores to (97.92±3.12) scores ( F=4.145, P=0.042), and GETE decreased from (2.47±0.27) scores to (1.60±0.19) scores ( t=2.982, P=0.010). The actual value of FEV 1, FEV 1% pred, the maximum expiratory flow percentage of the predicted value (PEF% pred), forced expiratory flow at 25% (MEF 75), forced expiratory flow at 50% (MEF 50) and maximum midexpiratory flow (MMEF 75/25), increased from (1.96±0.12) L to (2.17±0.21) L ( F=0.425, P=0.789), (81.46±2.85)% to (82.64±1.55)% ( F=0.926, P=0.465), (82.05±3.58)% to (91.10±4.67)% ( F=1.909, P=0.128), (60.36±7.43)% to (76.94±4.65)% ( F=2.120, P=0.096), (52.72±3.75)% to (73.80±8.34)% ( F=3.140, P=0.047) and (60.05±8.47)% to (74.86±10.85)% ( F=7.860, P=0.010), respectively.FeNO decreased from 25.0 (14.5, 35.5) μg/L to 20.0 (18.5, 30.0) μg/L ( Z=-0.206, P=0.840). Transient headache was observed in 1 case and evanescent eruption in 2 cases during the treatment, respectively. Conclusions:Omalizumab can significantly improve the clinical manifestations, lung function indicators and quality of life of children with moderate and severe allergic asthma, and has good safety.Thus, it is expected to play an important role in the treatment of children with moderate-to-severe allergic asthma.

Secondary therapy-related acute megakaryocytic leukemia in childhood acute lymphocytic leukemia: report of one case and review of literature / 白血病·淋巴瘤

Objective:To explore the laboratory characteristics and diagnostic methods for therapy-related acute megakaryocytic leukemia (t-AMKL).Methods:The data of one child with acute lymphoblastic leukemia (ALL) in the Blood Disease Hospital of Chinese Academy of Medical Sciences & Peking Union Medical College in September 2014 was retrospectively analyzed. After inducing remission for more than 43 months, the child was diagnosed as t-AMKL.Results:After the diagnosis of ALL, the child was given chemotherapy with standard childhood ALL regimen. After 43 months, t-AMKL was diagnosed by comprehensive morphology, cytogenetics, and molecular biology. Bone marrow morphology showed that the proportion of primitive cells was 0.44; flow cytometry showed the phenotype was abnormal myeloid primitive cells; the pathology result showed that the abnormal cells weakly expressed CD42b and CD61; the electron microscopy showed platelet peroxidase (PPO)-positive and myeloperoxidase (MPO)-negative; the bone marrow immunohistochemistry showed the positive rate of CD41 was 34%; the child had a complex karyotype. After reviewing his medical history, he was diagnosed as t-AMKL.Conclusion:The t-AMKL is relatively rare, and it is helpful to improve the prognosis of patients by completing the relevant examinations for early diagnosis.

Changes of dopamine D2 receptor in patients with insomnia and its clinical significance / 中华核医学与分子影像杂志

Objective To explore the changes of dopamine D2 receptor in dopamine pathway in in-somnia patients and discuss its clinical significance. Methods From January 2016 to December 2016, 15 patients with insomnia (1 male, 14 females, age:(44.3±8.6) years) and 15 gender-/age-matched-healthy volunteers (control group;3 males, 12 females, age:(40.5±9.0) years) were included to undergo resting brain 11C-Raclopride PET/CT imaging. The D2 receptor binding potential (BPND) of the dopamine pathway was calculated by molecular imaging and kinetic analysis toolbox ( MIAKAT) software. The BP ND , Hamilton depression scale ( HAMD) , transient and graphics memory scale results were compared with two-sample t test and Mann-Whitney u test between the two groups. Pearson correlation analysis was used to evaluate the correlation between BPND(nucleus accumbens, caudate nucleus, putamen) and Pittsburgh sleep quality in-dex ( PSQI) , HAMD, course of disease, transient memory and graphical memory scale scores in the patient group. Results The BP ND in bilateral putamen, nucleus accumbens and left caudate nucleus of patients was lower than that of controls( left putamen:z=-2.717, right putamen:z=-2.883, both P<0.01;left nu-cleus accumbens:t=-2.269, right nucleus accumbens:t=-2.410, both P<0.05;left caudate nucleus:t=-2.632,P<0. 05), but the BPND level of right caudate nucleus was not significantly different(z=-0.850, P>0.05) . The scores of HAMD in the patient group were higher than those in control group ( t=10. 273, P<0. 01), while the scores of instantaneous memory (t=-4.888, P<0.01) and graphical memory scale (t=-2.624, P<0.05) were lower. There were significant negative correlations between the BP ND of bilateral nucleus ac-cumbens, caudate nucleus and putamen and the course of insomnia in the patient group ( r range:-0.761 to-0.682, all P<0.01) . Conclusion Patients with insomnia have abnormal neurotransmitter system of dopa-mine D2 and it may play a role in the pathogenesis of insomnia.

Abnormal functional connectivity of the cores that control rapid eye movement sleep in idiopathic rapid eye movement sleep behavior disorder / 中华神经科杂志

Objective To investigate the abnormal functional connectivity (FC) between the cores (including sublaterodorsal nucleus (SLD) and ventrolateral periaqueductal gray matter (vlPAG)) and the whole brain in rapid eye movement sleep behavior disorder (RBD) by resting state functional magnetic resonance imaging (rfMRI).Methods A total of 41 subjects recruited in the Department of Neurology,the People's Hospital of Zhengzhou University were enrolled in this study according to international diagnosis criteria,including 20 with idiopathic RBD (iRBD group) and 21 age,sex-matched normal controls (control group).All subjects were examined by Hoehn-Yahr Staging,cognitive tests and rfMRI.Resluts HoehnYahr staging score was 0(0,0) in the iRBD group,which showed no significant difference from that in the control group (0 (0,0),Z =-1.820,P =0.069).The scores of Rey Auditory Verbal Learning Test (AVLT) N1,AVLT N2,Symbol Digital Modalities Test,Rey-Osterrieth Complex Figure Test-Copy were 3.80 ± 1.67,5.10 ± 1.77,33.00(31.25,34.00) and 22.00(20.25,26.00) respectively in the iRBD group,which were significantly lower than that in the control group (4.95 ± 1.28,t =2.482,P =0.017;6.43±1.16,t =2.848,P=0.007;33.00(29.50,35.50),Z=-3.792,P=0.000;35.00(33.00,36.00),Z =-2.351,P =0.019) respectively.The scores of Trail Making Test 1 (86.5 (70.0,100.0))and Trail Making Test 2 (197.0(180.5,211.5)) in the iRBD group were significantly higher than that in the control group (66.0(49.0,91.5),112.0(99.5,173.0) respectively,Z=-2.373,P=0.018;Z =-3.105,P =0.002).Compared with the control group,the FC analysis showed reduced connections from the right SLD to the bilateral cingnlate gyrus (t =-4.173) and bilateral frontal gyrus (t =-2.965(left),-3.662(right)),from the vlPAG to the left precentral-postcentral gyrus(t =3.930),and from the vlPAG to the right frontal gyrus (t =4.141) in the iRBD.There was no statistically significant difference from the left SLD to the whole brain.Conclusion There were abnormal FCs from the SLD and vlPAG to cognitive and motor areas in RBD patients,perhaps leading to clinical RBD symptoms such as cognitive deterioration and movement disorder.

Analysis of characteristics of polysomnography in patients with multiple dreams / 中华神经科杂志

Objective To investigate the characteristics of sleep structures in patients with multiple dreams through the retrospective analysis of polysomnography in patients with multiple dreams,and to provide a theoretical basis for the treatment of multiple dreams.Methods Twenty-two cases with multiple dreams in Department of Neurology,the People's Hospital of Zhengzhou University from July 2015 to Ferbuary 2018 were included in multi-dream group and 12 healthy people in control group.The sleep parameters related to polysomnography during the visit were collected and recorded,and the differences between the two groups were compared.Resluts There was no statistically significant difference in apneahypopnea index,sleep latency,rapid eye movement (REM) sleep latency,slow-wave sleep ratio,and REM-arousal index between the two groups.Compared with the control group,sleep efficiency (73.46％ ± 12.41％ vs 90.43％ ± 4.42％,t=-4.555,P=0.000),REM period ratio (16.28％ ± 5.59％ vs 21.59％ ± 2.70％,t =-3.727,P =0.001) decreased in the multi-dream group;whereas ratio of light sleep (66.49％ ±9.97％ vs 59.85％ ±3.01％,t =2.966,P =0.006),awakening numbers (13.4 ±6.98 vs 6.08 ± 3.34,t =3.411,P =0.002),arousal index (20.11 ± 10.69 vs 11.82 ± 8.09,t =2.338,P =0.026),non-REM arousal index (20.22 ± 10.53 vs 12.08 ± 8.69,t =2.283,P =0.029) increased.Conclusion The sleep efficiency of patients with multiple dreams is reduced,and their perceived dreams may originate from light sleep periods.

Fatal familial insomnia preliminarily diagnosed as frontotemporal dementia: a case report and literature review / 中华神经科杂志

Objective To explore the clinical,imaging,genetic features in a case of fatal familial insomnia (FFI),and review related literatures.Methods A case of middle-aged woman diagnosed as frontotemporal dementia based on the preliminary manifestation of abnormal mental behavior was reported.The clinical features,imaging characteristics,electroencephalogram and polysomnogram of the patient were analyzed,and the blood samples from the patient and some of her familial members were collected for the sequencing of prion protein gene (PRNP).Results This patient was a middle-aged woman,whose clinical manifestations were abnormal mental behavior,rapid progressive dementia and intractable insomnia,abnormal night sleep behavior and laryngeal stridor.Brain MRI indicated frontotemporal lobe atrophy.Non-sleep disturbance was observed in polysomnography.The cerebrospinal fluid was negative for 14-3-3 protein.The results of PRNP sequencing revealed that the mutation of gene D178N/129M was detected.Conclusions Detection of PRNP plays an important role in the diagnosis of FFI.Patients suspected of FFI in clinic should be detected for genetic testing.Whether the frontotemporal lobe atrophy was caused by FFI or concurrent with FFI remains to be further verified.

Degree centrality analysis of brain functional network in insomnia disorder based on voxel / 中国医学影像技术

Objective To explore the changes of brain functional network in insomnia disorder (ID) during resting-state with voxel-based degree centrality (DC).Methods Forty-five subjects underwent resting-state fMRI scans,including 22 patients with ID (ID group) and 23 sex-,age-,and education-matched healthy volunteers (control group).The subjects' sleep quality and emotion state were assessed with Pittsburgh sleep quality index (PSQI),Hamilton anxiety scale and Hamilton depression scale.The resting-state fMRI data were analyzed with voxel-based DC.The intra-and inter-groups parameters were compared using t-test.Correlation analysis was performed between DC values of ID group and clinical parameters.Results Compared with control group,DC values increased in left parahippocampal gyrus,left hippocampus and bilateral precuneus (all P＜0.05),while decreased in left middle occipital lobe,left precuneus,left inferior frontal gyrus and left middle frontal gyrus in ID group (all P＜0.05).DC values of the left hippocampus in ID patients showed significantly negative correlation with the score of Pittsburgh sleep quality index (r=-0.46,P=0.047).Conclusion ID patients have abnormal DC distribution of brain functional network,therefore providing basis for understanding pathophysiological mechanisms of ID.

Non-displaceable binding potential changes of striatal dopamine D2 receptors in patients with first-episode major depressive disorder and the correlation with clinical features / 中华核医学与分子影像杂志

Objective To observe non-displaceable binding potential (BPND) changes of striatal dopamine D2 receptors(SDDR) in patients with first-episode major depressive disorder (MDD) using 11C-Raclopride PET/CT,and to analyze the relationship between BPND and Hamilton rating scale for depression (HAM-D).Methods From December 2014 to December 2015,patients with first-episode MDD and age/gender-matched healthy controls underwent brain MRI and 11C-Raclopride PET/CT in this prospective study.BPND of bilateral SDDR was calculated by molecular imaging and kinetic analysis toolbox (MIAKAT).BPND changes of bilateral SDDR and their relationship with HAM-D score were analyzed.Paired t test,two-sample t test and Pearson correlation analysis were used.Results A total of 20 MDD patients (8 males,12 females,average age: (32.80±9.76) years) and 20 healthy controls (9 males,11 females,average age:(29.25±6.93) years) were enrolled in this study.The 11C-Raclopride uptake in brain tissue of the MDD group and control group were mainly distributed in bilateral striatum,and very few 11C-Raclopride was distributed in bilateral cerebral cortex and cerebellum.In MDD group,the BPND level of bilateral SDDR had no statistical differences(t values: 0.69,0.35,both P>0.05),and similar results were found in the control group(t values: 0.28,0.24,both P>0.05).Compared with the control group,however,the MDD group had lower BPND level of bilateral SDDR(t values: 3.13-4.41,all P<0.05).The BPND of bilateral caudate nucleus and/or putamen D2 receptors was correlated with HAM-D total score,anxiety/somatization factor score,cognitive impairment factor score,retardation factor score and sleep disturbance factor score(r values: from-0.688 to-0.453,all P<0.05).Conclusions The binding potential of SDDR in patients with first-episode MDD is declined,and the BPND level of SDDR is correlated with symptoms of depression.The abnormality of SDDR may be an important molecular mechanism of the abnormality of midbrain-striatal dopamine reward circuits in MDD patients.

Functional connectivity between the substantia nigra and the motor cortex in the rapid eye movement sleep behavior disorder / 中华神经科杂志

Objective To investigate the abnormal functional connectivity (FC) between the substantia nigra (SN) and the brain motor area in rapid eye movement sleep behavior disorder (RBD) by Unified Parkinson′s Disease Rating Scale (UPDRS), Hoehn-Yahr Scale and resting state functional magnetic resonance imaging (rfMRI).Methods A total of 34 subjects (14 with RBD (RBD group), 12 with Parkinson′s disease (PD group), and 8 age, sex-matched normal controls (control group)) recruited in the Department of Neurology, Henan Provincial People′s Hospital from 2014 to 2015 were enrolled in this study according to international diagnosis criteria.All subjects were examined by UPDRS, Hoehn-Yahr Scale and rfMRI.Results UPDRS scores and Hoehn-Yahr staging were 0.00 (0.00, 3.75) and 0.00 (0.00, 0.50) respectively in the RBD group, which were significantly different from that in the PD group (30.5 (18.75, 33.00) and 1.75 (1.50, 2.50), respectively;Z=-3.782, P0.05 and Z=1.10, P>0.05).The FC analysis showed alterations from the right SN to bilateral cerebellum respectively among the three groups (Fright cerebellum=12.975, Fleft cerebellum=6.144;P0.05).Conclusions There was abnormal FC from the SN to motor areas in RBD patients, and partial alterations were similar as PD patients.rfMRI provided an evidence that RBD might be presymptom of PD.

Clinicopathologic characteristics of plasma cell myeloma with marrow fibrosis / 中华病理学杂志

Objective@#To study the clinicopathologic features of plasma cell myeloma(PCM) with bone marrow fibrosis (MF).@*Methods@#The clinicopathologic data of 175 cases of newly diagnosed PCM patients were retrospectively analyzed. Based on reticular fiber staining, these cases were divided into PCM-MF and non-PCM-MF groups.@*Results@#Sixty-three cases were PCM-MF(36%), 112 were non-PCM-MF (64%). No statistical difference in gender, age, hemoglobin level, platelet counts, the classification of immunoglobulin, ISS staging, immunohistochemical phenotypes and genetic features was found between PCM-MF and non-PCM-MF groups (P>0.05). Compared to non-PCM-MF group, lactate dehydrogenase (LDH)level and renal impairmentrate were higher in PCM-MF group (P<0.05). The degree of bone marrow hyperplasia, the percentage of myeloma cells and cells with plasmablastic morphology were significantly higher in PCM-MF group(P<0.05).@*Conclusion@#The higher LDH level, renal impairment rate, and more significant bone marrow hyperplasia, proliferation of plasma cells and plasmablastic myeloma cells infiltration indicate poor prognosis of PCM-MF patients.

Clinical Value of Echocardiography on Aortic Valvuloplasty in Low-age Pediatric Patients With Congenital Aortic Valve Stenosis / 中国循环杂志

Objective: To evaluate the clinical value of echocardiography in aortic valvuloplasty (AVP) in the low-age pediatric patients with congenital aortic valve stenosis. Methods: We retrospectively studied 39 low-age (at median of 23 months) patients with congenital aortic valve stenosis who received aortic valve repair in our hospital for their echocardiography information, and statistically analyzed the main indicator changes by 4 time points as pre-operation and 1 week, 1-3 months, 6-12 months after the operation respectively. Results: In our study, the bicuspid to tricuspid valve ratio was approximately at 5.5/1 and 2 patients died during peri-operative period. Compared with pre-operative time point, Doppler aortic valve peak velocity (Vmax ) and the mean aortic transvalvular pressure gradient (MPG) were reduced accordingly, for Vmax: (4.30 ± 0.73) m/s vs (2.65 ± 0.78) m/s, (2.93 ± 0.63) m/s, (3.01 ± 0.83) m/s,P<0.01, for MPG: (45.78 ± 15.19) mmHg vs (18.24 ± 10.08) mmHg, (21.01 ± 10.08) mmHg, (22.31 ± 13.41) mmHg. Compared with pre-operative time point, left ventricular ejection fractions (LVEF) were similar in 3 post-operative time points. Compared with 1 week post operative time point, left ventricular end-diastolic anteroposterior diameter (LVEDD) was increased at 6-12 months post-operative time point, the relative wall thickness (RWT) was decreased, bothP<0.05, and aorta valve regurgitation (AR) was increasedP<0.01. Pearson correlation analysis showed that aortic annulus (AA) inner diameter was positively related to LVEDD (r= 0.648,P<0.01), negatively related to Vmax (r= -0.205,P<0.05) and RWT was positively related to Vmax (r= 0.196,P<0.05). There were 6 patients with pre-operatively decreased LVEF, 1 of them died and the rest 5 with elevated LVEF at 6-12 months post-operative period,P<0.05. Conclusion: Echocardiograghy could be used as the ifrst choice of imaging method for diagnosing congenital aortic valve stenosis, it has the important role for in-operative monitoring and post-operative evaluation of AVP in relevant patients.

Pathological diagnosis of pediatric Burkitt lymphoma involving bone marrow / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate pathologic and differential diagnostic features of pediatric Burkitt lymphoma (BL).</p><p><b>METHODS</b>A total of 20 cases of pediatric BL were retrospectively reviewed for their clinical and pathologic profiles. Bone marrow aspiration specimens were available in all cases and bone marrow biopsies were available for immunohistochemical study in 18 cases. Flow cytometry study was available in 16 cases. MYC translocation by FISH method was performed in 11 cases.</p><p><b>RESULTS</b>Atypical lymphocytes with cytoplasmic vacuoles were found in bone marrow smears in all 20 cases and peripheral blood films in all 19 available cases. The bone marrow biopsies showed infiltration by uniform medium-sized atypical lymphocytes with multiple small nucleoli but without the starry-sky pattern in all 18 cases. Immunohistochemistry showed the following results in all 18 cases: positive for CD20, PAX-5, CD10, CD34 and TdT, but negative for bcl-2 and CD3 with Ki-67 > 95%.Flow cytometry showed CD19+CD20+CD10+FMC7+CD22+TdT-CD3- in 16 cases, including κ+ in 8 cases, λ+ in 7 cases, and κ-λ- in 1 case. MYC gene rearrangement by FISH was observed in 10 of the 11 cases.</p><p><b>CONCLUSIONS</b>The histopathology of BL is distinct, including atypical lymphocytes with cytoplasmic vacuoles in bone marrow aspirate, lack of starry-sky patternin bone marrow biopsy. Generally, the diagnosis should be made with a combined immunophenotype and FISH approach. Pediatric BL must be distinguished from DLBCL and B-cell lymphoma, unclassifiable, which has intermediate features between DLBCL and Burkitt lymphoma.</p>

The clinic and pathologic significance of plasma cell myeloma with CCND1 / 中华血液学杂志

<p><b>OBJECTIVE</b>To study the clinical and pathologic features of multiple myeloma(MM) with CCND1.</p><p><b>METHODS</b>Retrospectively analyzed the clinical and pathologic profiles of 158 patients with MM from 2010 to 2013. The clinical and morphologic features of bone marrow aspiration, biopsy and immunophenotypic analysis which was carried out by flow cytometry and immunohistochemistry were analyzed in all patients with MM respectively. CCND1 translocation was studied by FISH method in all cases. Classical cytogenetic studies of bone marrow were performed in 24 cases whose CCND1 was positive.</p><p><b>RESULTS</b>In the 158 patients with MM, CCND1 was detected in 31 patients (19.6%）. In 31 patients, type IgA, IgD, IgG, IgM, light-chain only and nonsecretory MM were 4 cases,4 cases,11 cases,1 case, 6 cases and 5 cases respectively. A high incidence of CCND1 was observed in IgD and nonsecretory MM comparied with IgA and IgG respectively (P<0.05). but no statistical significance was reached between κ and λ type patients (P=0.627). The morphology of plasma cell in bone marrow biopsies were small Lymphocyte- Like 24 cases,mature plasma cell 6 cases and immature plasma cell 1 case. Immunophenotype of all 31 cases was CD38⁺CD138⁺CD19⁻CD45⁻, (CD56⁺ in 11 cases, CD20⁺ in 9 cases, CD117⁺ in 3 cases. MM with CCND1 showed a strong association with CD20 expression, the lack of CD56 expression. Immunohistochemistry showed positive for cyclinD1 in 22 cases.</p><p><b>CONCLUSION</b>A high incidence of CCND1 was detected in the IgD and nonsecretory MM, and correlated with Small Lymphocyte- Like, higher positive rate of CD20, cyclinD1 and the lack of CD56 expression. MM with CCND1 must be distinguished from LPL and other mature B cell lymphomas which have plasmacytoid differentiation.</p>

Application of Color Doppler Ultrasound in Diagnosis of Deep Vein Thrombosis of Lower Limbs for Pulmonary Embolism following Spinal Cord Injury / 中国康复理论与实践

@# Objective To study the value of color Doppler ultrasound in diagnosis of deep vein thrombosis (DVT) of lower extremity and pulmonary embolism (PE) in spinal cord injury (SCI). Methods 60 PE patients (PE group) and 35 SCI patients without PE (control group) received color Doppler ultrasound examination for DVT of lower extremity. Results PE group included 36 SCI patients (PE-SCI group) and 24 no-SCI patients (PE-no-SCI group). There were 15 cases with lower extremity thrombosis in PE-SCI group, and 9 cases in PE-no-SCI group (P>0.05), while there were 5 cases in the control group. There was significantly different in lower extremity thrombosis between PE group and the control group (P<0.01). In PE group, the detection rate was not significantly different between acute PE (detected 11 cases out of 37 cases) and chronic PE (detected 10 cases out of 23 cases) (P>0.05). 31 cases were rechecked as lower extremity venous valve regurgitation and calf muscle vein dilation (51.7%) in PE group while 8 cases in the control group (22.9%) (P<0.01). Conclusion There is not significantly different in the detection rate of DVT of lower extremity in PE patients with and without spinal cord injury, which are higher than in the patients without PE. Color Doppler ultrasound is necessary to check DVT in acute and chronic PE patients.

Primary bone marrow diffuse large B cell lymphoma: three case reports and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To report the diagnosis, differential diagnosis and treatment of three rare cases of primary bone marrow diffuse large B cell lymphoma (DLBCL), and to improve the recognition of this disease.</p><p><b>METHODS</b>The clinical characteristics, therapeutic course and the outcome of these patients were reviewed. Meanwhile, a series of examinations including morphology, flow cytometry, immunohistochemistry and molecular biology of bone marrow samples were also performed.</p><p><b>RESULTS</b>These three patients who were old at the onset age (56, 60 and 70 years old), primarily revealed as abnormal blood count and experienced an aggressive course of disease. Physical and imaging examination showed no enlargement of lymph node, liver and spleen, the patients were finally diagnosed as primary bone marrow DLBCL by bone marrow morphology, flow cytometry and immunohistochemistry analyses. They were treated with rituximab combined chemotherapy, which achieved a complete response, but still need longer follow-up to further evaluate their survival.</p><p><b>CONCLUSION</b>Primary bone marrow DLBCL was encountered rarely in clinical practice, and this is the first report in China. Further investigation of pathogenesis and therapeutic strategies of this rare disease was warranted.</p>

Dorsolateral prefrontal cortex connectivity in patients with primary insomnia: evidence from resting state functional magnetic resonance imaging / 中华神经科杂志

Objective To investigate cognitive impairment mechanism by studying dorsolateral prefrontal cortex connectivity in patients with primary insomnia.Methods Forty patients with primary insomnia and 50 healthy subjects from the Department of Neurology,People's Hospital of Zhengzhou University during the period April 2011 through April 2013 were included.The World Health OrganizationUniversity of California Los Angeles Auditory Verbal Learning Test (WHO-UCLA AVLT) and the digital pin test were applied to evaluate the subjects' word study ability and vigilance.Resting state functional magnetic resonance imaging was used to observe the connectivity of dorsolateral prefrontal cortex.Results The Pittsburgh Sleep Quality Index (2.00 (1.00,3.00)) and the Hamilton Anxiety Scale scores (13.00 (11.25,15.75)) of primary insomnia patients were significantly higher than that of healthy controls (11.00(9.00,13.00),1.00 (0,2.00),Z=-5.517,Z=-5.525,P＜0.01).Digital pin test efficiency (60.03％ ± 13.95％ vs 66.32％ ± 13.73％,t =2.142,P＜0.05) and WHO-UCLA word learning (10.11 ± 2.29 vs 11.95 ± 2.42,t =-3.493,P ＜ 0.01) of primary insomnia patients were significantly lower than that of healthy controls.Compared to the healthy controls,the right dorsolateral prefrontal cortex of primary insomnia patients exhibited decreased functional connectivity of the right prefrontal lobe (-2.610 3 ± 0.172 6,t =-3.504,P ＜ 0.05).The left dorsolateral prefrontal cortex of primary insomnia patients exhibited increased functional connectivity of the bilateral insular lobes and right prefrontal lobe (2.8204±0.326 5,2.371 7 ±0.106 6,2.492 6 ±0.052 8,t =4.032,t =3.340,t =3.037,P ＜0.05).Conclusions The ability of WHO-UCLA word study and the digital pin test efficiency have been shown to decline in patients with primary insomnia.The possible mechanism of cognitive impairment may be the abnormal dorsolateral prefrontal cortex connectivity in patients with primary insomnia.

A two-year follow-up for Chinese patients with abdominal aortic aneurysm undergoing open/endovascular repair / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>A number of studies have demonstrated the rates of overall and aneurysm-related mortality and morbidity in Western populations. The cardiovascular risk factors influencing postoperative outcome have been also reported. Until recently, little has been known about the prognosis in this patient cohort in the Chinese population. We evaluated the independent predictors of mortality and morbidity in abdominal aortic aneurysm (AAA) patients undergoing elective surgical treatment and emphasized whether the coronary artery revascularization could have any effect on the overall mortality and morbidity in patients following the current guideline recommendation.</p><p><b>METHODS</b>A total of 386 patients (174 women) undergoing surgery in Beijing Anzhen Hospital from January 2008 to June 2010 were enrolled (mean age (70.6±10.5) years). Kaplan-Meier curves were constructed to compare the mortality and morbidity of AAA patients with coronary artery revascularization and those without. A Cox proportional hazards model was constructed to identify clinical factors associated with two-year outcomes. The primary outcomes were death from any cause, the pre-specified morbidity was re-hospitalization for pulmonary conditions, congestive heart failure, angina, ischemic/hemorrhagic stroke.</p><p><b>RESULTS</b>During the two-year follow-up, 34 patients died and 65 experienced re-hospitalization with pulmonary conditions, congestive heart failure, angina, or ischemic/hemorrhagic stroke. Kaplan-Meier survival analysis showed that the AAA patients with cardiac revascularization had no higher incidence of overall mortality and major morbidity than those without (log-rank test P = 0.35 and P = 0.40, respectively). Cox proportional hazards regression analysis showed that level of lowdensity lipoprotein (HR, 4.06; 95% CI: 1.19-18.7, P = 0.027) and AAA size (HR, 2.18; 95% CI: 1.28-11.65, P = 0.036) were independently associated with the incidence of overall mortality. Long-term use of angiotensin converting enzyme inhibitors, statins, AAA size and systolic blood pressure were independent predictors of the secondary pre-specified outcomes.</p><p><b>CONCLUSIONS</b>Coronary artery revascularization following the guideline recommendations did not increase the mortality and morbidity of Chinese with AAA who were undergoing repair. Absence of angiotensin converting enzyme inhibitors and statins, AAA size, and systolic blood pressure were powerful predictors of the clinical events.</p>

Application of Color Doppler Ultrasound in Diagnosis of Deep Vein Thrombosis of Lower Limbs for Pulmonary Embolism following Spinal Cord Injury / 中国康复理论与实践

Objective To study the value of color Doppler ultrasound in diagnosis of deep vein thrombosis (DVT) of lower extremity and pulmonary embolism (PE) in spinal cord injury (SCI). Methods 60 PE patients (PE group) and 35 SCI patients without PE (control group) received color Doppler ultrasound examination for DVT of lower extremity. Results PE group included 36 SCI patients (PE-SCI group) and 24 no-SCI patients (PE-no-SCI group). There were 15 cases with lower extremity thrombosis in PE-SCI group, and 9 cases in PE-no-SCI group (P>0.05), while there were 5 cases in the control group. There was significantly different in lower extremity thrombosis be-tween PE group and the control group (P0.05). 31 cases were rechecked as lower extremity venous valve regurgitation and calf muscle vein dilation (51.7%) in PE group while 8 cases in the control group (22.9%) (P<0.01). Conclusion There is not significantly different in the detection rate of DVT of lower extremity in PE patients with and without spinal cord injury, which are higher than in the patients without PE. Color Doppler ultrasound is necessary to check DVT in acute and chronic PE patients.

Incidence of cardiovascular damage in ankylosing spondylitis patients and the related risk factors / 中华风湿病学杂志

Objective To survey the incidence of cardiovascular damage in ankylosing spondylitis (AS) patients and explore the related risk factors.And to establish the risk prediction models for the future.Methods The electrocardiogram (ECG) and cardiac ultrasonography (UCG) of 122 AS patients (group A)in the department of rheumatology of Peking Union Medical College Hospital from 2001 to 2011 were analyzed.Fifty-nine AS patients with abnormal ECG and/or UCG findings consisted of the study group (group A1),the other 63 AS patients without cardiovascular damage were the control group (group A2).We selected 100healthy controls (group B).All healthy controls had ECG and UCG tests.The ECG and UCG findings of the group A1 were analyzed,the clinical manifestations and laboratory results were compared between group A1and A2.A univariate analysis and multivariate Logistic regression analysis were carried out to determine the risk factors related to cardiovascular damage in AS.Results The prevalence rate of cardiovascular damage in AS patients was 48.4％ (59/122).While the prevalence of ECG and or UCG abnormality in healthy controls was 12.0％ (12/100),the difference between the two groups was statistically significant (P＜0.05).In group A1,the main findings in ECG were abnormal sinus node function,high voltage in left ventricle and ST-T changes,other findings were atrioventricular block,branch bundle block and premature beats.In group A1,the main findings in UCG were valvular diseases and arterial root widening,other abnormal findings were pericardial effusion,left ventricular diastolic dysfunction and mild pulmonary hypertension.The level of ESR,CRP,RBC,HGB,blood glucose,the number of peripheral joint involvement,the severity of sacroiliac arthritis and the age were significantly different between group A1 and A2 (P＜0.05).Multivariate Logistic regression analysis showed that age and CRP were risk factors for cardiovascular damage in AS patients.Conclusion The prevalence of cardiovascular damage in ankylosing spondylitis is relatively high.Age and CRP are the risk factors contributing to cardiovascular damage in AS patients.