Influencing factors for inadequate bowel preparation of colonoscopy of different colonic segments / 中华消化内镜杂志

Objective:To investigate the influencing factors for inadequate bowel preparation of colonoscopy.Methods:A total of 677 patients who underwent colonoscopy at Peking Union Medical College Hospital from December 2021 to January 2023 were recruited, and all patients underwent standardized bowel preparation by using 3 L polyglycol electrolyte powder with fractional dose. The quality of bowel preparation was assessed by Boston bowel preparation scale, and the influencing factors for inadequate bowel preparation were analyzed by logistic regression analysis.Results:The rate of inadequate bowel preparation was 31.5% (213/677). Among the patients with inadequate bowel preparation, 85.4% (182/213) inadequate bowel preparation was only in proximal colon, 14.1% (30/213) was in both proximal and distal colon, and 0.5% (1/213) was only in distal colon. Inadequate bowel preparation in distal colon and total colon were combined into inadequate bowel preparation in distal colon. The results of logistic regression analysis showed that inadequate bowel preparation in proximal colon was more likely to occur in men ( P=0.001, OR=2.253, 95% CI: 1.399-3.629), outpatients ( P<0.001, OR=4.175, 95% CI: 2.410-7.231), those with no family history of colorectal cancer ( P=0.001, OR=2.117, 95% CI: 1.365-3.284), and diagnostic colonoscopy ( P=0.003, OR=1.978, 95% CI: 1.261-3.102). And spinal disease ( P=0.044, OR=7.430, 95% CI: 1.051-52.511), outpatients ( P<0.001, OR=135.577, 95% CI: 29.135-630.883),non-compliance of dietary requirements ( P=0.006, OR=4.772, 95% CI: 1.576-14.453), adverse reaction during bowel preparation ( P=0.015, OR=4.341, 95% CI: 1.329-14.179), no family history of colorectal cancer ( P=0.003, OR=7.110, 95% CI: 1.912-26.438), and poor last stool character ( P=0.001, OR=25.922, 95% CI: 3.779-177.832) were risk factors for inadequate bowel preparation in distal colon. Conclusions:The inadequate bowel preparation of colonoscopy mainly occurs in proximal colon, and the risk factors for the inadequate bowel preparation vary in different colonic segments. Therefore, the specific interventions should be performed according to the character of different colon segments to improve the quality of bowel preparation.

Association of Galectin-3 with atrial fibrillation / 中国综合临床

Objective:To investigate the effect of galactose lectin 3 (Gal-3) on the pathogenesis of atrial fibrillation.Methods:This study adopts a case-control study method. 55 patients with non valvular atrial fibrillation (atrial fibrillation group) admitted to the First People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from February to July 2019 were selected, and another 55 healthy individuals who underwent physical examination at our hospital during the same period were selected as the control group. Compare the general data and levels of various laboratory indicators between two groups, including blood routine, fasting blood glucose, blood lipids, liver and kidney function, and plasma Gal-3. Analyze the influencing factors of atrial fibrillation and the predictive value of plasma Gal-3 levels for the onset of atrial fibrillation. The measurement data with normal distribution and the measurement data converted to normal distribution after taking natural logarithm are expressed in xˉ± s. The comparison between the two groups is performed by independent sample t test; The measurement data of non normal distribution is represented by [ M ( Q1, Q3)], and Wilcoxon signed rank sum test is used for inter group comparison; The counting data is represented by examples (%), and the comparison between groups is conducted using χ 2 test. The influencing factors of atrial fibrillation were analyzed using logistic regression analysis. Results:The age, NLR, and blood creatinine levels in the atrial fibrillation group were higher than those in the control group [(71.16±9.17) years vs (60.71±10.11) years, (2.32±0.85) vs (1.74±0.81), (74.18±21.61) μmol/L vs (64.69±18.30) μmol/L, t-values are 5.68, 3.66, 2.48, P-values are <0.001, <0.001, 0.015], total cholesterol, HDL-C, LDL-C Albumin and eGFR water were on average lower than those in the control group [(4.31±1.67) mmol/L vs (5.13±0.78) mmol/L, (0.96±0.21) mmol/L vs (1.21±0.32) mmol/L, (2.35±0.65) mmol/L vs (3.04±0.62) mmol/L, (39.58±3.83) g/L vs (44.66±5.61) g/L, (94.84±29.22) mL/(min·1.73 m 2) vs (111.77±21.51) mL/(min·1.73 m 2)] ,The t-values are 3.30, 4.87, 5.69, 5.54, 3.46, and the P-values are 0.001,<0.001,<0.001,<0.001, 0.001, respectively. The plasma Gal-3 levels in the atrial fibrillation group were higher than those in the control group [(12.79±4.24)] μg/L vs (7.31±2.28) μg/L], the difference was statistically significant ( t=8.43, P<0.001), and the plasma Gal-3 level in the persistent atrial fibrillation group was higher than that in the paroxysmal atrial fibrillation group [(14.03±3.95) μg/L vs (11.51±4.21) μg/L], the difference was statistically significant ( t=2.29, P=0.026). The results of multivariate logistic regression analysis showed that after excluding other factors, Gal-3 remained an independent influencing factor for atrial fibrillation (odds ratio=1.66, 95% confidence interval: 1.29-2.12, P<0.001). Conclusions:Plasma Gal-3 is an influencing factor for the onset of atrial fibrillation. After excluding other factors, Gal-3 remains an independent influencing factor for atrial fibrillation, with an increase of 1 μg/L in Gal-3 increases the risk of atrial fibrillation by 1.66 times.

Correlation between TCM syndrome and clinical indicators and disease activity in Sjogren's syndrome / 国际中医中药杂志

Objective:To explore the correlation between Traditional Chinese Medicine (TCM) syndrome types of Sj?gren syndrome (SS) and blood test parameters, immunological function and disease activity.Methods:A retrospective cross-sectional study was conducted. The clinical data of 242 SS inpatients in the Rheumatology and Immunology Department of Jiangsu Province Hospital of TCM from February 2021 to June 2022 were analyzed retrospectively. We compared the general data (gender, age, course of disease, BMI), blood parameters [WBC, hemoglobin (Hb), PLT, neutrophil count (NEUT), lymphocyte count(LYMPH), neutrophil/lymphocyte ratio (NLR)], immunological indicators (globulin, IgG, IgA, IgM, rheumatoid factor (RF), anti-SSA antibody, anti-SSB antibody, anti-Ro-52 antibody) .The distribution difference of disease activity [Disease Activity Index of Sjogren's syndrome (ESSDAI) and Patient Report Index of Sjogren's syndrome (ESSPRI)], the correlation between each syndrome type and blood routine parameters, immunological indicators and inflammatory indicators was analyzed by binary logistic regression.Results:They were divided into 82 cases of qi yin deficiency syndrome, 61 cases of yin deficiency and fluid deficiency syndrome, 59 cases of yin deficiency and blood stasis syndrome, 32 cases of yin deficiency and heat toxin syndrome, and 8 cases of other syndrome types. Because the number of other syndrome types was small, they were not included in this study. Logistic regression analysis showed that the positive rate of anti SSA antibody was negatively correlated with IgM [ OR (95% CI)=0.570 (0.407, 0.798)] ( P<0.01). The positive rates of anti SSB antibody and anti Ro-52 antibody were negatively correlated with LYMPH [ OR (95% CI)=0.445 (0.223, 0.886), 0.457 (0.224, 0.932), respectively] ( P<0.05). The positive rates of anti SSB antibody and anti Ro-52 antibody were positively correlated with IgG [ OR (95% CI)=1.171 (1.034, 1.325), 1.159 (1.014, 1.325), respectively] ( P<0.05). Qi Yin deficiency syndrome was positively correlated with WBC [ OR (95% CI)=2.590 (1.120, 5.987)] ( P<0.05), and negatively correlated with LYMPH [ OR (95% CI)=0.090 (0.017, 0.470)] and IgA [ OR (95% CI)=0.728 (0.553, 0.959)] ( P<0.05). Yin deficiency and fluid deficiency syndrome were negatively correlated with PLT [ OR (95% CI)=0.991 (0.984, 0.998)], ESSPRI [ OR (95% CI)=0.705 (0.506, 0.983)], ESSDAI [ OR (95% CI)=0.716 (0.534, 0.960)] ( P<0.05). Yin deficiency and blood stasis syndrome was positively correlated with IgA [ OR (95% CI)=1.184 (1.028, 1.363)] ( P<0.05), and negatively correlated with anti SSB antibody positive rate [ OR (95% CI)=0.247 (0.093, 0.659)] ( P<0.05). Yin deficiency heat toxin syndrome was positively correlated with IgA [ OR (95% CI)=1.368 (1.037, 1.803)] ( P<0.05), and negatively correlated with anti SSB antibody positive rate [ OR (95% CI)=0.278 (0.085, 0.909)] ( P<0.05). Conclusion:The level of immunoglobulin, inflammatory index and disease activity of yin deficiency and blood stasis syndrome and yin deficiency and heat toxin syndrome are high, and blood system damage and exocrine gland disease are easy to occur, which can provide clinical basis for the combination of disease and syndrome differentiation and treatment of SS.

Preclinical and early clinical studies of a novel compound SYHA1813 that efficiently crosses the blood-brain barrier and exhibits potent activity against glioblastoma

Glioblastoma (GBM) is the most common and aggressive malignant brain tumor in adults and is poorly controlled. Previous studies have shown that both macrophages and angiogenesis play significant roles in GBM progression, and co-targeting of CSF1R and VEGFR is likely to be an effective strategy for GBM treatment. Therefore, this study developed a novel and selective inhibitor of CSF1R and VEGFR, SYHA1813, possessing potent antitumor activity against GBM. SYHA1813 inhibited VEGFR and CSF1R kinase activities with high potency and selectivity and thus blocked the cell viability of HUVECs and macrophages and exhibited anti-angiogenetic effects both in vitro and in vivo. SYHA1813 also displayed potent in vivo antitumor activity against GBM in immune-competent and immune-deficient mouse models, including temozolomide (TMZ) insensitive tumors. Notably, SYHA1813 could penetrate the blood-brain barrier (BBB) and prolong the survival time of mice bearing intracranial GBM xenografts. Moreover, SYHA1813 treatment resulted in a synergistic antitumor efficacy in combination with the PD-1 antibody. As a clinical proof of concept, SYHA1813 achieved confirmed responses in patients with recurrent GBM in an ongoing first-in-human phase I trial. The data of this study support the rationale for an ongoing phase I clinical study (ChiCTR2100045380).

Analysis of IQSEC2 gene variant in a child with X-linked mental retardation / 中华医学遗传学杂志

OBJECTIVE@#To analyze the clinical phenotype and genetic variants of a child with X-linked mental retardation caused by IQSEC2 gene mutation, and provide reference for the diagnosis of the disease.@*METHODS@#The child was subjected to next generation sequencing (NGS), and the diagnosis was made by taking consideration of her clinical characteristics.@*RESULTS@#The child has presented with global developmental delay, particularly in fine motor skill and language development, in addition with intellectual disability. Genetic testing revealed that she has harbored a heterozygous c.1861dup variant of the IQSEC2 gene, which was not detected in either parent.@*CONCLUSION@#The de novo c.186ldup variant of the IQSEC2 gene probably underlay the X-linked mental retardation in this child. Above finding has, expanded the spectrum of IQSEC2 gene mutations and provide a basis for the diagnosis of similar cases.

Research progress on neurodevelopmental disorders in children with Duchenne muscular dystrophy / 中华实用儿科临床杂志

Duchenne muscular dystrophy (DMD) is a disease inherited in an X-linked recessive pattern, which is caused by the pathogenic mutation of the gene encoding Dystrophin.An increasing number of studies have confirmed the high risk of neurodevelopmental disorders in children with DMD, and that related comorbidities have distinct clinical characteristics.In this article, the research progress on neurodevelopmental disorders in children with DMD was reviewed to clarify the prevalence, clinical characteristics and high-risk factors of neurodevelopmental disorders in children with DMD.DMD therapy teams should pay attention to the evaluation, interpretation and early intervention of neurodevelopmental disorders in clinic practice, so as to improve the life quality of DMD children and help them to be-tter integrate into the society.

Clinical application of the new classification criteria in children with Miller Fisher syndrome and clinical characteristics analysis of different types of Miller Fisher syndrome / 中华实用儿科临床杂志

Objective:To investigate the clinical application of the new classification criteria in children with Miller Fisher syndrome (MFS), and to analyze clinical characteristics of different types of MFS.Methods:Clinical data of MFS patients hospitalized in the Department of Neurology, Beijing Children′s Hospital, Capital Medical University from January 2015 to December 2019 were collected and analyzed retrospectively, including demographic characteristics, clinical symptoms, neurological examination findings, laboratory examination data, diagnosis and treatment, and prognosis.The counting data was described by percentage, and the measurement data was described by median.Results:A total of 23 patients were included in the research, including 14 males and 9 females, with a median age of 4 years and 8 months.There were 3 cases of pure MFS; 5 cases of incomplete MFS, including 1 case of acute ptosis and 4 cases of acute ataxia neuropathy; 15 cases of overlapping syndrome, including 13 cases of MFS/Guillain Barre syndrome (GBS), 1 case of MFS/pharyngocervical brachial variant GBS(PCB GBS)and 1 case of MFS/GBS/Bickertaff brainstem encephalitis (BBE). In addition to Ⅲ, Ⅳ and Ⅵ cranial nerve palsy, 11 cases had the involvement of other cra-nial nerves, including 2 cases in pure MFS, 8 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Autonomic nervous dysfunction occurred in 6 cases.Respiratory muscle paralysis occurred in 6 cases, including 5 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Graded by the Hughes scoring system (HG score), 3 cases with pure MFS were graded 4 points; 1 case with acute ptosis was graded 0; 3 cases with acute ataxia neuropathy were graded 2 points, and the other one was graded 3 points; 1 case with MFS/PCB GBS was graded 3 points; 10 cases with MFS/GBS were graded 4 points, 1 case was graded 3 points, and the other 2 cases were graded 2 points; 1 case with MFS/GBS/BBE was graded 4 points.Twenty-two patients were treated with intravenous immunoglobulin.The HG of all patients at discharge decreased at varying degree, which was graded 0 at 6 months of follow-up.Conclusions:The clinical application of the new diagnostic classification method is helpful to the accurate diagnosis of different types of MFS.More than half of MFS cases will develop into the overlapping syndrome.The overlapping of MFS and GBS or BBE is prone to the involvement of cranial nerves except for the external ophthalmic muscles, autonomic nerve dysfunction and respiratory muscle paralysis.The disease course of MFS varies, and its diagnosis should be comprehensively made.All cases of MFS in this study have a satisfactory prognosis.

Clinical value of serum albumin in the evaluation of left atrial thrombosis in patients with nonvalvular atrial fibrillation / 中华老年医学杂志

Objective:To investigate the relationship between serum albumin(SA)levels and left atrial thrombosis(LAT)in elderly patients with nonvalvular atrial fibrillation(NVAF), and to evaluate the clinical value of SA in predicting LAT in elderly NVAF patients.Methods:Clinical data of 180 elderly patients with NVAF undergone transesophageal echocardiography(TEE)in our hospital were retrospectively analyzed.According to whether there was thrombosis in the left atrium, patients were divided into the thrombus group(n=42)and the non-thrombus group(n=138). Logistic regression was used to analyze factors related to left atrial appendage thrombosis in NVAF patients.The receiver operating characteristic(ROC)curve was used to evaluate the value of serum albumin levels in predicting LAT formation in elderly NVAF patients.Results:The duration of AF was longer in the thrombus group than in the non-thrombus group(all P<0.05). C-reactive protein(CRP)levels and platelet(PLT)counts were higher, and SA levels and the international normalization ratio(INR)were lower in the thrombus group than in the non-thrombus group(all P<0.05). The left atrial diameter(LAD)was larger in the thrombus group than in the non-thrombus group( P<0.05). Multiple Logistic regression analysis showed that the duration of AF and CRP levels were independent risk factors for LAT( P<0.05), and SA levels and INR were independent protective factors for LAT( P<0.05). ROC curve results showed that the area under the curve(AUC)of SA in predicting LAT was 0.778(95% CI: 0.711-0.837, P<0.001). Conclusions:Low serum albumin levels are closely related to LAT in NVAF patients and can be regarded as a predictor of LAT.Serum albumin levels should be monitored in clinical practice to reduce the incidence of stroke events in patients with AF.

The CnB1 p.D102A variant is linked to dilated cardiomyopathy via impaired Calcineurin activity.

BACKGROUND: The role of calcineurin (protein phosphatase 2B (PP2B)) in the pathogenesis of human dilated cardiomyopathy (DCM) has not been fully elucidated. We determined the potential involvement of calcineurin in the pathogenesis of DCM caused by mutations in CnB1, a subunit of calcineurin. METHODS: By whole-exome sequencing, we identified a new CnB1 variant in a Han Chinese proband with cardiomyopathy from a 3-generation family with 2 normal individuals and 3 individuals with familial dilated cardiomyopathy. The potential pathogenic variant was validated by Sanger sequencing. We performed functional and mechanistic experiments in a CnB1-knockin (KI) mouse model and at the cellular level. RESULTS: We detected a rare heterozygous CnB1 variant (p.D102A) in a proband with dilated cardiomyopathy. This variant was localized to the EF hand 3 region of CnB1, where no variants have been previously reported. KI mice harboring the p.D102A variant exhibited decreased cardiac function and cardiac dilatation. Immunoblotting, RT-PCR and immunofluorescence results showed decreased cardiomyocyte size and heart failure-related protein expression. A calcineurin activity assay demonstrated decreased calcineurin activity in the KI mice, accompanied by the decreased ability of CnB1 to bind CnA. CONCLUSIONS: CnB1 p.D102A is a disease-associated variant that confers susceptibility to cardiac dilatation. This variant is associated with impaired calcineurin activity and a subsequent decrease in the ability of CnB1 to bind CnA.

Early chest computed tomography to diagnose COVID-19 from suspected patients: A multicenter retrospective study

ObjectiveThe purpose of this study is to distinguish the imaging features of COVID-19 with other chest infectious diseases and evaluate diagnostic value of chest CT for suspected patients. MethodsAdult suspected patients aged>18 years within 14 days who underwent chest CT scan and reverse-transcription polymerase-chain-reaction (RT-PCR) tests were enrolled. The enrolled patients were confirmed and grouped according to results of RT-PCR tests. The data of basic demographics, single chest CT features, and combined chest CT features were analyzed for confirmed and non-confirmed groups. ResultsA total of 130 patients were enrolled with 54 cases positive and 76 cases negative. The typical CT imaging features of positive group were ground glass opacity (GGO), crazy-paving pattern and air bronchogram. The lesions were mostly distributed bilaterally, close to the lower lungs or the pleura. When features combined, GGO with bilateral pulmonary distribution and GGO with pleural distribution were more common, of which were 31 cases (57.4%) and 30 cases (55.6%) respectively. The combinations were almost presented statistically significant (P<0.05) except for the combination of GGO with consolidation. Most combinations presented relatively low sensitivity but extremely high specificity. The average specificity of these combinations is around 90%. ConclusionsThe combinations of GGO could be useful in the identification and differential diagnosis of COVID-19, which alerts clinicians to isolate patients for treatment promptly and repeat RT-PCR tests until incubation ends.

A comparative multi-centre study on the clinical and imaging features of comfirmed and uncomfirmed patients with COVID-19

BackgroundPrevious studies had described the differences in clinical characteristics between ICU and non-ICU patients. However, seldom study focused on confirmed and unconfirmed groups. Our aim was to compare clinical and imaging characteristics of COVID-19 patients outside Hubei province between confirmed and unconfirmed group. MethodsWe retrospectively enrolled 163 consecutive adult patients with suspected COVID-19 from three tertiary hospitals in two provinces outside Hubei province from January 12, 2020 to February 13, 2020 and the differences in epidemiological, clinical, laboratory and imaging characteristics between the two groups were compared. ResultsThis study enrolled 163 patients with 62 confirmed cases and 101 unconfirmed cases. Most confirmed patients were clustered (31, 50.0%) and with definite epidemiological exposure. Symptoms of COVID-19 were nonspecific, largely fever and dry cough. Laboratory findings in confirmed group were characterized by normal or reduced white blood cell count, reduced the absolute value of lymphocytes, and elevated levels of C-reactive protein (CRP) and accelerated Erythrocyte sedimentation rate (ESR). The typical chest CT imaging features of patients with confirmed COVID-19 were peripherally distributed multifocal GGO with predominance in the lower lung lobe. Compared with unconfirmed patients, confirmed patients had significantly higher proportion of dry cough, leucopenia, lymphopenia and accelerated ESR (P<0.05); but not with alanine aminotransferase, aspartate aminotransferase, D-dimer, lactic dehydrogenase, and myoglobin (P>0.05). Proportion of peripheral, bilateral or lower lung distribution and multi-lobe involvement, GGO, crazy-paving pattern, air bronchogram and pleural thickening in the confirmed group were also higher (P<0.05). ConclusionsSymptoms of COVID-19 were nonspecific. Leukopenia, lymphopenia and ESR, as well as chest CT could be used as a clue for clinical diagnosis of COVID-19.

Current status and progress of 2019 novel coronavirus pneumonia / 中华危重病急救医学

Recently, the 2019 novel coronavirus (2019-nCoV) pneumonia outbroke in Wuhan and rapidly spread to all over China and even the world. Because of the strong infectivity and various clinical symptoms, it has brought certain difficulties to the epidemic prevention and control. Currently there is no specific drug for 2019-nCoV. Previous drugs used to treat other coronaviruses may be effective, but further clinical trials remain needed. We reviewed literature on the epidemiology, etiology, clinical manifestations, imaging manifestations, laboratory examination, diagnosis, complications, treatment and outcome of 2019-nCoV pneumonia.

Clinical features and laboratory characteristics of eight patients with subacute sclerosing panencephalitis / 中华神经科杂志

Objective:To investigate the clinical and laboratory characteristics of subacute sclerosing panencephalitis (SSPE).Methods:The clinical, laboratory and electroencephalogram (EEG) data of eight patients with SSPE who admitted to the Department of Neurology, Beijing Children's Hospital, Capital Medical University, from May 2014 to February 2019 were retrospectively analyzed and followed up.Results:Four of the patients were male and four were female, who aged from two years and seven months to 13 years and five months with a median onset age of five years and six months. All of the eight cases had disease onset with progressive mental and physical regression, then developed periodic myoclonic seizures at the course of 11 days to 11 months. Video EEG examinations showed persistent generalized periodic complex waves with long interval (3-20 s). The IgG titers of measles virus in blood and cerebrospinal fluid of all cases were significantly increased. There was no significant abnormality in blood/urine metabolism screening nor head magnetic resonance imaging for the first time. Five cases performed head magnetic resonance imaging again, in which two cases with deepening hemispheric sulcus, two cases with cerebral white matter signal abnormalities. Antiepileptic drugs, gamma globulin, adrenocortical hormone and antiviral drugs were used after diagnosis though all were ineffective. All patients presented progressive deterioration. During the follow-up period of three months to two years and seven months, four patients died, of which three patients died at the time of five months, one year and two months, two years and six months after onset respectively, and the other one was unknown.Conclusions:The diagnostic clues of SSPE are progressive mental and physical regression, recurrent myoclonic seizures during period Ⅱ, as well as the extensive periodic complex waves of EEG. It is necessary to detect measles virus IgG antibody in blood and cerebrospinal fluid to make a definite diagnosis. There is no specific treatment for SSPE and its prognosis is very poor.

Current status and progress of 2019 novel coronavirus pneumonia / 中华危重病急救医学

Recently, the 2019 novel coronavirus (2019-nCoV) pneumonia outbroke in Wuhan and rapidly spread to all over China and even the world. Because of the strong infectivity and various clinical symptoms, it has brought certain difficulties to the epidemic prevention and control. Currently there is no specific drug for 2019-nCoV. Previous drugs used to treat other coronaviruses may be effective, but further clinical trials remain needed. We reviewed literature on the epidemiology, etiology, clinical manifestations, imaging manifestations, laboratory examination, diagnosis, complications, treatment and outcome of 2019-nCoV pneumonia.

Clinical analysis of 13 cases with acute pandysautonomia / 中华实用儿科临床杂志

Objective:To summarize the clinical data of patients with acute pandysautonomia (APD) and discuss the treatment and prognosis of them.Methods:A total of 13 patients with APD in the Department of Neurology, Beijing Children′s Hospital, Capital Medical University, from January 2010 to December 2019, were investigated retrospectively.The general data, clinical symptoms, autonomic nerve examination and function test, laboratory examination, treatment and follow-up were collected and analyzed.Results:There were 4 males and 9 females in 13 patients with APD, with an average age was 8 years and 5 months (3 years and 8 months to 12 years and 5 months ). The average course of disease was 94.5 d (14-410 d). The common initial symptoms were gastrointestinal motility disorder (11 cases), dysuria (3 cases), and upright syncope/vertigo (3 cases). During the course of the disease, all the patients manifested with gastrointestinal motility disfunction and dyshidrosis, glands involvement and orthostatic hypotension in 12 cases, abnormal pupil in 9 case and urinary retention in 7 case.Other symptoms included fatigue in 9 cases, emotional disorder in 4 cases, limb weakness in 2 cases, and sensory disturbance in 2 cases.All the patients were treated with intravenous immunoglobulin (IVIG), and 3 cases combined with glucocorticoid.Six patients with severe gastrointestinal symptoms were treated with intravenous nutrition; 4 patients were fed with jejunum, 3 cases of whom returned to normal diet within 1-12 months, and 1 patient was followed up for 5 years and 2 months.Hyponatremia was found in 7 cases, which recovered in 2-30 d. Nine cases were followed up for 1 month to 9 years.Seven cases were normal in daily work and study, with satisfactory nutritional status, stable mood and no relapse.Conclusions:The clinical manifestations of APD are varied.The initial symptoms are gastrointestinal motility disorders, orthostatic hypotension, urinary retention and hyponatremia.Individualized multi-disciplinary comprehensive management for symptoms, especially the comprehensive treatment of gastrointestinal motility disorders, management of postural hypotension, and the urinary system diagnosis and individualized treatment of can shorten the length of hospital stay and improve the prognosis effectively.

Inhibition of ebracteolatain A in the proliferation of breast cancer cells by interfering with PKD1-mediated MEK/ERK and PI3K/AKT signaling pathways / 药学实践杂志

Objective Breast cancer is one of the deadliest malignancies in the world. ebracteolatain A (EA) is a kind of acetylphloroglucinol extracted from ebracteolatain. To explore the specific mechanism of EA inhibiting the proliferation of breast cancer cell MCF-7, so as to provide a new approach for the clinical treatment of breast cancer. Methods EA with different concentrations were added to breast cancer cell MCF-7 to detect changes in PKD1 protein expression. The plasmid with overexpressed PKD1 was constructed and transfected into cells, and the mRNA and protein expression levels of PKD1 were detected by real-time fluorescence quantitative PCR and Western Blot assay. CCK-8 assay was used to detect changes in cell proliferation capacity. Western Blot assay was used to detect the expression level of PKD1 and its related signaling pathways. Results EA inhibited the expression of PKD1 protein in breast cancer cells with a dose-dependent manner (P< 0.05). When transfected with the overexpressed plasmid, PKD1 was significantly increased in mRNA and protein levels (P<0.001). At the same time, PKD1 overexpression significantly reversed inhibition of EA on MCF-7 proliferation (P<0.001). It was confirmed by signaling pathway analysis that EA might affect the proliferation ability of breast cancer cells by inhibiting PKD1-mediated MEK/ERK and PI3K/AKT signaling activity (P<0.05). Conclusion EA could inhibit the proliferation of breast cancer cells by regulating PKD1-mediated MEK/ERK and PI3K/AKT signaling pathways.

Clinical features of 19 children with Bickerstaff brainstem encephalitis / 中华儿科杂志

Objective@#To summarize the clinical features of Bickerstaff brainstem encephalitis (BBE) in children.@*Methods@#In this retrospective study, data of 19 patients with BBE (11 males and 8 females) were collected from Department of Neurology, Beijing Children′s Hospital from October 2015 to January 2018. The clinical features, treatment and prognosis were analyzed.@*Results@#The onset age of BBE ranged from 1 year and 8 months to 12 years and 11 months. There were 18 cases with preceding infection. The most common infection was upper respiratory tract infection (9 cases), followed by simple fever (5 cases). The most common initial neurological symptoms were lethargy or disturbance of consciousness (8 cases), followed by limb weakness (5 cases). There were 6 cases of simple BBE and 13 cases of BBE overlapping Guillain-Barré syndrome (GBS). Besides the characteristic triad of altered mental status, ataxia, and ophthalmoplegia, there were other symptoms including convulsion (5 cases), diplopia (3 cases), nystagmus (7 cases), facial muscular weakness (7 cases),bulbar palsy (13 cases) and autonomic nerve symptoms (9 cases). Hypo or areflexia was seen in 16 cases. Positive Babinski′s signs were seen in 8 cases. Hyponatremia was present in 10 cases in whom 4 showed severe hyponatremia. Albumin-cytological dissociation of cerebrospinal fluid was seen in 10 cases. The autoimmune antibodies were examined in all 19 patients. Anti-ganglioside antibodies including anti-GM1 IgG antibody was positive in 2 patients and one of whom was also found with positive anti-GD1b IgG antibody. Anti-GQ1b IgG antibody was present in 2 patients. Electromyography was performed in 14 cases and 8 cases, who were all BBE overlapping GBS, showed neurological damage. A total of 16 cases were monitored by video electroencephalography and 8 cases showed slow waves of background. In addition to, interictal focal discharge was detected in 2 cases. T2 fluid-attenuated inversion recovery (FLAIR) sequence abnormal signals were detected in 3 of 18 cases performed brain magnetic resonance imaging (MRI), and lesions involved with brainstem, basal ganglia, thalamus, cerebellum, corpus callosum and cerebral cortex. Lesions involved cervical and thoracic spinal cord were found in 1 out of 11 cases for whom spinal cord MRI was performed. All of the 4 cases who underwent enhanced MRI of spinal had partial nerve roots enhancement. All of the 19 patients received 1 to 2 courses of intravenous immunoglobulin therapy, and 2 cases also received plasma exchange. Fifteen cases received steroid therapy. The following-up period ranged from 3 months to 2.5 years. Two cases were lost to follow-up. Twelve cases achieved a full recovery within 3 months. Three cases recovered within 6 months. One case still had slight limb weakness and ataxia after 1 year and 8 months of follow-up, and another case had left autonomic nerve symptoms in the follow-up of 2 years and 3 months. Both of them were BBE overlapping GBS.@*Conclusions@#Children′s BBE is similar to that in adults, and is frequently found overlapped with GBS. Furthermore, it is sometimes accompanied by central nervous system demyelination disease. The antiganglioside antibodies are not often detectable. Immunoglobulin therapy could usually achieve good response. The prognosis of simple BBE is good in most situations. For BBE overlapping GBS, the more severe the limb weakness during the peak of disease is, the slower the recovery would be.

Level of high-mobility group box 1 in patients with chronic hepatitis B and liver cirrhosis and its clinical significance / 临床肝胆病杂志

ObjectiveTo investigate the correlation of serum high-mobility group box 1 (HMGB1) level with hepatic inflammatory activity, liver fibrosis degree, and liver function parameters in chronic hepatitis B patients. MethodsA total of 73 patients with chronic hepatitis B and liver cirrhosis were enrolled as subjects. Liver biopsy was performed to determine inflammatory activity and liver fibrosis degree, liver function parameters and serum HMGB1 level were measured, and the correlation between HMGB1 level and other indices was analyzed. The t-test was used for comparison between two groups, and a linear correlation analysis was performed to investigate the correlation between two indices. ResultsThe patients with chronic hepatitis B and liver cirrhosis had a significantly higher HMGB1 level than the healthy control group (29.46±7.54 ng/ml vs 16.86±3.48 ng/ml, t=5.668, P＜0.01). The G3-G4 group had a significantly higher HMGB1 level than the G1-G2 group (t=4.441, P＜0.01), while there was no significant difference in HMGB1 level between the S1-S2 group and the S3-S4 group (t=0.658, P＞0.05). Serum HMGB1 level was positively correlated with alanine aminotransferase (r=0.256 6, P=0028 4) and aspartate aminotransferase (r=0.471 9, P＜0.000 1), while it was not correlated with albumin or total bilirubin (both P＞005). ConclusionSerum HMGB1 level is closely correlated with hepatic inflammatory activity.

Convulsion, disturbance of consciousness, fever / 中华实用儿科临床杂志

The child was a preschool girl who was admitted for the first time due to " convulsions,disturbance of consciousness and fever for 2 days".The girl was diagnosed as acute disseminated encephalomyelitis (ADEM).After immunotherapy,the girl recovered.During the course of hormone reduction,the cranial magnetic resonance imaging showed aggravated,and neurological symptoms were followed.There was a transient decrease of blood cells.After repeat hormone treatment,the girl was improved again,but the intracranial lesions still showed progressive aggravation tendency.The course of central nervous system demyelinating in this patient was unknown.When her younger sibling prestentd fever,hepatosplenomegaly,blood cell decreased and was diagnosed with familial haemophagocytosis syndrome(FHPS),through case discussion and gene testing,the girl was finally diagnosis "central nervous system involvement at the onset of FHPS".The case suggests unexplained central nervous system demyelinating lesions,regardless of whether there are abnormalities of blood system,should pay attention to haemophagocytosis syndrome.Positive immunotherapy should be given after diagnosis,and stem cell transplantation should be given as soon as possible to improve prognosis.

Salmonella virulence gene analysis in poisoning food,slaughtering and commercial samples / 中国人兽共患病学报

We investigated the carrying status of the virulence genes of Salmonella from different sources in Shijiazhuang City,China,to provide the basic data for the further risk assessment of Salmonella.A total of 186 isolates of Salmonella from different sources were collected and identified serotypes in the area of Shijiazhuang from 2011 to 2016.PCR was performed for eight virulence genes (invA,sopE,agfA,spvR,hilA,stn,pefA,shdA).These Salmonella bacteria were detected in 13 kinds of serotypes.Enteritidis is a significant advantage of the group.The above 8 virulence genes were analyzed,and the virulence genes hilA,stn and invA were the most frequently carried,their respective carrying rate were 90.3％ (168/186),86.6％ (161/186) and 82.8％ (154/186) respectively.We found the virulence genes of Salmonella from different sources were different.It is necessary to take measures to strengthen the food hygiene supervision and prevention and control of the storage and sale of raw poultry stalls in the morning market in Shijiazhuang area.