Objective:To investigate the safety and feasibility of the CHESS endoscpic ruler (CHESS ruler), and the consistency between the measured values and the interpretation values by endoscopic physician experience.Methods:From January 2021 to January 2022, a total of 105 liver cirrhosis patients with portal hypertension were prospectively enrolled from General Hospital, Xixia Branch Hospital, Ningnan Hospital of People′s Hospital of Ningxia Hui Autonomous Region (29 cases), and the First People′s Hospital of Yinchuan (25 cases), General Hospital of Ningxia Medical University (18 cases), Wuzhong People′s Hospital (10 cases), the Fifth People′s Hospital of Ningxia Hui Autonomous Region (10 cases), Shizuishan Second People′s Hospital (6 cases), Yinchuan Second People′s Hospital (5 cases), and Zhongwei People′s Hospital (2 cases) 8 hospitals. The clinical characteristics of all the patients, including gender, age, nationality, etiolog of liver cirrhosis, and Child-Pugh classification of liver function were recorded. A big gastroesophageal varices was defined as diameter of varices ≥5 mm. Endoscopist (associated chief physician) performed gastroscopy according to the routine gastroscopy procedures, and the diameter of the biggest esophageal varices was measured by experience and images were collected, and then objective measurement was with the CHESS ruler and images were collected. The diameter of esophageal varices of 10 randomly selected patients (random number table method) was determined by 6 endoscopists (attending physician or associated chief physician) with experience or measured by CHESS ruler. Kappa test was used to test the consistency in the diameter of esophageal varices between measured values by CHESS ruler and the interpretation values by endoscopic physician experience.Results:Among 105 liver cirrhosis patients with portal hypertension, male 65 cases and female 40 cases, aged (54.8±12.2) years old, Han nationality 82 cases, Hui nationality 21 cases and Mongolian nationality 2 cases. The etiology of liver cirrhosis included chronic hepatitis B (79 cases), alcoholic liver disease (7 cases), autoimmune hepatitis (7 cases), chronic hepatitis C (2 cases), and other etiology (10 cases). Liver function of 32 cases was Child-Pugh A, Child-Pugh B 57 cases, and Child-Pugh C 16 cases. All 105 liver cirrhosis patients with cirrhotic portal hypertension were successfully measured the diameter of gastroesophageal varices by CHESS ruler, and the success rate of application of CHESS ruler was 100.0% (105/105). The procedure time from the CHESS ruler into the body to the exit of the body after measurement was (3.50±2.55) min. No complications happened in all the patients during measurement. Among 105 liver cirrhosis patients with cirrhotic portal hypertension, 96 cases (91.4%) were recognized as big gastroesophageal varices by the endoscopists. Totally 93 cases (88.6%) were considered as big gastroesophageal varices by CHESS ruler. Eight cases were recognized as big gastroesophageal varices by the endoscopist, however not by the CHESS ruler; 5 cases were recognized as big gastroesophageal varices by the CHESS ruler, but not by the endoscopists; 4 cases were not recognized as big gastroesophageal varices both by the endoscopists and CHESS ruler; 88 cases were recognized as big gastroesophageal varices both by the endoscopists and CHESS ruler. The missed diagnostic rate of big gastroesophageal varices by the endoscopists experience was 5.4% (5/93), and the Kappa value of consistency coefficient between the measurement by the CHESS ruler and the interpretation by endoscopists experience was 0.31 (95% confidence interval 0.03 to 0.60). The overall Kappa value of consistency coefficient by 6 endoscopists measured by CHESS ruler in big gastroesophageal varices diagnosis was 0.77 (95% confidence interval 0.61 to 0.93).Conclusion:As an objective measurement tool, CHESS ruler can make up for the deficiency of subjective judgment by endoscopists, accurately measure the diameter of gastroesophageal varices, and is highly feasible and safe.

Objective A simple,specific and rapid LC-MS/MS method was established to determine flumatinib and its two major metabolites in human plasma for clinical therapeutic drug monitoring.Methods The determination was performed on an ACQUITY UPLC HSS T3 column(2.1 mm×50 mm,1.8 μm)with mobile phases consisting of acetonitrile and 10 mmol·L-1 ammonium formate(containing 0.1%formic acid)with gradient elution at the flow rate of 0.5 mL·min-1.The elution time was 6 min.The temperature of the column was 38℃.The ion source was electrospray ion source and the scanning mode was multiple reaction monitoring scanning in positive ion mode.Results The mass concentrations of flumatinib and its metabolites(flumatinib M1 and flumatinib M3)have a good linear relationship within the concentration range investigated.The precision and stability of the method are good.The precision is less than 15%,and the relative deviation is within±15%.The extraction recoveries of flumatinib and its metabolites approach nearly 100%.Conclusion The method is simple and sensitive,and can accurately determine the plasma concentration of flumatinib and its metabolites,providing a basis for clinical rational drug use.

Exercise therapy is an important part of the comprehensive treatment of peripheral vascular diseases, including walking, high-intensity intermittent exercise, resistance exercise and other forms. This article reviews the intensity (exercise load, severity of claudication during exercise, increase of exercise volume) and duration (duration of each exercise, exercise rest ratio, weekly exercise frequency, duration of treatment course) of exercise, and points out that medical and nursing staff should formulate personalized "exercise prescriptions" according to patients' tolerance, and provide personalized nursing guidance to patients, so that patients with peripheral vascular diseases can obtain great benefits in exercise therapy.

Objective:To summarize the genotype and phenotype of epilepsy in patients with interferon regulatory factor 2 binding protein-like (IRF2BPL) gene variants.Methods:Data of 6 epilepsy patients with IRF2BPL gene variants seen from May 2017 to September 2020 in the Department of Pediatrics of Peking University First Hospital were retrospectively collected. The clinical characteristics and genetic test results were analyzed.Results:A total of 6 patients with IRF2BPL gene variants (1 boy and 5 girls) were identified. The age of seizure onset was from 3.5 to 7.0 months. Epileptic spasms were observed in 6 patients, tonic seizures and tonic-spasms were observed in 1 patient and focal seizure was observed in 1 patient. All 6 patients presented with developmental delay, 5 patients presented with hypotonia, and 2 patients presented with dysphagia. Microcephaly,nystagmus,chorea and athetosis were observed in 1 patient. The electroencephalography (EEG) showed slow background activity in 2 patients. Hypsarrhythmia was observed in all 6 patients. Focal epileptic discharges were observed in 2 patients. Epileptic spasms were monitored in all 6 patients. Focal seizure and tonic-spasm were monitored in 2 patients respectively. The brain magnetic resonance imaging (MRI) showed cerebral atrophy and dysplasia of the corpus callosum in 1 patient, delayed myelination in 2 patients and normal in 3 patients. Two patients had missense variants c.1280C>T/p.L474F and c.1420C>T/p.S427L, 3 patients had frameshift variants c.232delG/p.V78Sfs*73, c.244del/p.A82Pfs*70 and c.283-308del/p.Ala95Thrfs*29, 1 patient had non-frameshift deletion variant c.1453-c.1455delTTC/p.F485del, and all of the 6 cases had de novo variants. All patients were diagnosed with infantile spasms. The last follow-up age ranged from 1 year to 3.8 years. Four patients achieved seizure-free and 2 patients still had frequent seizures after the treatment with antiepileptic drugs (adrenocorticotropic hormone, topiramate, and vigabatrin).Conclusions:IRF2BPL gene variants are mainly de novo. The age of seizure onset is mainly in infancy, and epilepsy and developmental delay are the main clinical manifestations. Infantile spasm is the main phenotype, some patients have hypotonia and dysphagia. Cerebral atrophy can be observed in a few patients.

Objective:To analyse the epidemic features and programs of control on tuberculosis (TB) in China from 1990 to 2017 to provide references and evidence on prevention and control of the disease.Methods:We used data from the Global Burden of Disease Study 2017 to analyse the trends of incident and death cases of TB in China from 1990 to 2017.Results:In 2017, there were an estimated 831.0 thousand (age-standardized incidence: 54.18 per 100 000 population) incident cases and 39.3 thousand (age-standardised mortality: 2.17 per 100 000 population) deaths of TB in the country. The incident cases and deaths of TB decreased by 51.05 % and 76.24 % compared with the numbers in 1990, respectively. The average annual declining rates on incident cases and deaths of TB were 2.61 % and 5.18 %, respectively, from 1990 to 2017. The number of incident cases of TB decreased from 833.6 thousand in 2016 to 831.0 thousand in 2017 (decreased by 0.31 %). The number of deaths of TB decreased from 40.7 thousand in 2016 to 39.3 thousand in 2017 (decreased by 3.44 %). The number of incident cases and deaths of drug-sensitive TB showed a declining trend from 1990 to 2017. However, the number of incident cases and deaths showed first increased and then decreased trends for both multidrug-resistant TB (MDRTB) and extensively drug-resistant TB (XDRTB) in the same period. The number of incident cases of XDRTB increased from 2 979 in 2016 to 3 018 in 2017, with an increasing rate by 1.32 %. The number of deaths of XDRTB increased from 819 in 2016 to 829 in 2017, with an increase rate by 1.22 %. Conclusions:China made substantial progress in reducing both the TB incidence and mortality from 1990 to 2017 but the rate of decline became slow in the later years. We noticed that the increase of TB caused by XDR-TB had been increasing which called for special attention.

Objective: To analyze the epidemiological characteristics, trend and related factors of tuberculosis patients that delayed for care, in Wuhan from 2008 to 2017. Methods: Data regarding tuberculosis (TB) patients was collected from the tuberculosis management information system (TMIS), a part of the China information system for disease control and prevention from 2008 to 2017. A total of 64 208 tuberculosis patients, aged 0 to 95 years were included for the analysis. Unconditional logistic regression method was used to estimate those factors that associated with this study. Results: Days of delay among TB patients appeared as M=10 (P₂₅-P₇₅: 3-28) day, in Wuhan, 2008-2017. The prevalence of the delay was 52.5% (33 703/64 208), presenting a downward trend from 2008 to 2017 (trend χ²=10.64, P<0.001), but the proportions of women and ≥65 year-olds were gradually increasing. Results from the multivariate logistic regression analysis showed that factors as: patients living far away from the city vs. near the city (OR=1.29, 95%CI: 1.25-1.35), and age above 45 years vs. younger than 25 years (the age 45-64 years group vs. aged less than 25 years group, OR=1.22, 95%CI: 1.15-1.29; the age 65 or above group vs. aged less than 25 years group, the OR=1.30, 95%CI: 1.22-1.39) were under higher risk on the delay of seeking care. Occupation, way of case-finding and classification of tuberculosis patients also appeared as influencing factors on this issue. Conclusions Prevalence on the delay of care was 52.5% among tuberculosis patients in Wuhan, 2008-2017, but with an annual decrease. Attention should be paid to female, wrinkly or elderly tuberculosis patients regarding the delay of care on TB, in Wuhan.

Objective: To investigate the electroclinical findings in epilepsy children with epileptic negative myoclonus (ENM) restricted to the lower limb as the first seizure type. Methods: Each retrieved electroencephalogram record performed between March 2011 and March 2018 at the Department of Pediatrics of Peking University First Hospital was searched with "midline" . There were 302 records of 175 patients with "benign" or "functional" midline spikes. A retrospective review of each patient′s hospital record was performed. Thirteen patients had ENM restricted to the lower limb as the first seizure type. The clinical and electroencephalogram characteristics of them were analyzed. Results: Thirteen patients manifested ENM restricted to the lower limb as the first seizure type, diagnosed as benign childhood focal epilepsy with vertex spikes (BEVS). Six patients had ENM as the first and only seizure type during the short-time follow-up. Among them, there were 1 male and 5 females. The age at seizure onset was (2.5±0.7) years. One of them had electrical status epilepticus during sleep (ESES) identified on electroencephalogram at theage of 4 years and 8 months. The last follow-up age was (3.8±1.5) years. The remaining 7 patients developed nocturnal focal motor seizures. Among them, there were 4 males and 3 females. The age at seizure onset was (3.5±0.7) years. Two of them were diagnosed as BEVS evolving into benign childhood epilepsy with centrotemporal spikes (BECTS) and 5 were diagnosed as BEVS concurring with BECTS. The age at focal seizures was (4.1±0.6) years. The interval ranged from 1 month to 1 years. Six of 7 patients had electrical ESES with the age of (5.2±1.0) years. All had developmental regression, further diagnosed as atypical benign partial epilepsy (ABPE). The median age at last follow-up was 5.9 years. Five of 13 patients had repeated electroencephalogram records at our apartment, showing that epileptiform discharges in midline regions were significantly reduced either in frequency or amplitude with the improvement of ENM restricted to the lower limb and that independent epileptiform discharges in Rolandic regions from midline regions were noticed with the onset of nocturnal focal seizures. Conclusions ENM restricted to the lower limb has a close association with vertex (midline) epileptiform discharges. ENM restricted to the lower limb as the first seizure type is a peculiar phenomenon of BEVS. Some patients could evolve into BECTS or overlap with BECTS, and further into ABPE. The age of seizure onset in BEVS with ENM restricted to the lower limb as the first symptom is a little earlier than in BECTS. Ignorance of the close association between midline spikes and ENM restricted to the lower limb may lead to misdiagnosis of these patients.

Objective: To summarize the clinical and genetic features of β-propeller protein-associated neurodegeneration (BPAN). Methods: The clinical data of 17 patients with BPAN with WDR45 gene variants were retrospectively collected at Children’s Hospital of Fudan University, Peking University First Hospital, Capital Institute of Pediatrics, Shengjing Hospital of China Medical University and Shanghai Children's Hospital from June 2016 to December 2018, and their clinical manifestations, electroencephalogram, neuroimaging and genetics were analyzed. Results: Seventeen cases (13 females, 4 males), aged 1.1-8.8 years, were included. The median age of seizure onset was 14.5 months, from 3 months to 24 months of age, manifested with epileptic spasm in 6 cases and focal seizures in 5 cases. Eight patients had only one seizure type and 8 patients had two or more seizure types. Nine patients had complete remission of seizures. All 16 patients with seizures had developmental delay before the seizure onset, of whom 13 patients had moderate to severe seizures. The brain magnetic resonance imaging (MRI) was abnormal in 13 patients, including cerebral atrophy (10 cases) and thinning of the corpus callosum (9 cases). The brain magnetic susceptibility weighted imaging (SWI) in preschool stage showed prominent T2 hypointense signals in bilateral globus pallidus and brainstem ventral in two cases. Five seizure types (spasm, focal, absence, myodonic and generalized tonic clonic seizures)were found on ictal electroencephalogram(EEG) recordings. Compared to female patients(17(6-24) months of ege), male cases had earlier seizure onset (3, 4, 5, 18 months of age) . All patients had de novo variations in WDR45(6 nonsense, 4 frameshift, 3 missense and 4 splicing variations), with hemizygous variants in 3 males, mosaic variants in a male and heterozygous variants in 13 females, within which 5 variations had not been reported (c.977-1C>T,c.976+1G>C,c.10C>T,c.806del and c.110T>C). Conclusions The patients with BPAN have profound developmental delay and are vulnerable to seizures. The male patients with BPAN tend to have more severer clinical phenotype than females. Early brain SWI could facilitate the timely diagnosis of this disease.

Objective: To compare the visual quality after topography-guided customized femtosecond laser-assisted in situ keratomileusis (FS-LASIK) and wavefront-optimized FS-LASIK treatment in myopic eyes. Methods: A non-randomized controlled clinical study was performed.Seventy-eight eyes of 39 myopic patients undergoing FS-LASIK in Beijing Tongren Hospital from October 2016 to February 2017 were enrolled in this study and divided into two groups according to each patient's opinion, with matched demography between the two groups.Topography-guided customized FS-LASIK was performed on 42 eyes of 21 myopia in the topography-guided group, and wavefront-optimized FS-LASIK was performed on 36 eyes of 18 patients in the wavefront-optimized group.Visual acuity, refractive error, higher order aberrations (HOAs) and contrast sensitivity(CS) were compared between the two groups before and 6 months after surgery.Written informed consent was obtained from each patient before the operation.This study protocol was approved by Ethic Committee of Beijing Tongren Hospital (No.TRECKY2014-026). Results: The postoperative uncorrected visual acuity (UCVA) levels were eaqual to or better than the preoperative best corrected visual acuity (BCVA) in 95.2% patients in the topography-guided group, and in 94.4% patients in the wavefront-optimized group 6 months after surgery, respectively.There were no significant differences in the spherical equivalent, sphere refraction and cylinder refraction between the two groups (all at P>0.05). The amount of induced coma was significantly lower in the topography-guided group than that in the wavefront-optimized group ([0.07±0.22]μm vs.[0.22±0.16]μm) at 6 mm pupil.LogCS improved under the 12.0 c/d in the background of mesopic in the topography-guided group and decreased under the 18.0 c/d both in the background of mesopic and mesopic + glare in the wavefront-optimized group 6 months after surgery, with signifcant differences between them (all at P<0.05). LogCS values under 6.0, 12.0 and 18.0 c/d in the background of mesopic and 18.0 c/d in the background of mesopic+ glare in the topography-guided group were significantly higher than those in the wavefront-optimized group 6 months after surgery, with significant differences between the two groups (all at P<0.05). Conclusions Topography-guided FS-LASIK has lower higher-order aberrations and better CS than wavefront-optimized FS-LASIK.

To summarize the clinical and genetic features of β?propeller protein?associated neurodegeneration (BPAN). Methods The clinical data of 17 patients with BPAN with WDR45 gene variants were retrospectively collected at Children's Hospital of Fudan University, Peking University First Hospital, Capital Institute of Pediatrics, Shengjing Hospital of China Medical University and Shanghai Children's Hospital from June 2016 to December 2018, and their clinical manifestations, electroencephalogram, neuroimaging and genetics were analyzed. Results Seventeen cases (13 females, 4 males), aged 1.1-8.8 years, were included. The median age of seizure onset was 14.5 months, from 3 months to 24 months of age, manifested with epileptic spasm in 6 cases and focal seizures in 5 cases. Eight patients had only one seizure type and 8 patients had two or more seizure types. Nine patients had complete remission of seizures. All 16 patients with seizures had developmental delay before the seizure onset, of whom 13 patients had moderate to severe seizures. The brain magnetic resonance imaging (MRI) was abnormal in 13 patients, including cerebral atrophy (10 cases) and thinning of the corpus callosum (9 cases). The brain magnetic susceptibility weighted imaging (SWI) in preschool stage showed prominent T2 hypointense signals in bilateral globus pallidus and brainstem ventral in two cases. Five seizure types (spasm, focal, absence, myodonic and generalized tonic clonic seizures) were found on ictal electroencephalogram(EEG)recordings. Compared to female patients(17(6-24) months of ege), male cases had earlier seizure onset (3, 4, 5, 18 months of age). All patients had de novo variations in WDR45 (6 nonsense, 4 frameshift, 3 missense and 4 splicing variations), with hemizygous variants in 3 males, mosaic variants in a male and heterozygous variants in 13 females, within which 5 variations had not been reported (c.977?1C>T,c.976+1G>C,c.10C>T,c.806del and c.110T>C). Conclusions The patients with BPAN have profound developmental delay and are vulnerable to seizures. The male patients with BPAN tend to have more severer clinical phenotype than females. Early brain SWI could facilitate the timely diagnosis of this disease.