Urine glycosaminoglycans of two patients (full brother and sister )with Sanfilippo's syndrome were analysed by electrophoresis, enzymatic or chemical treatment and ion-exchange chromatography. Heparin sulfate is the main glycosaminoglycan accouting for 70% and 58% of urine glycosaminoglycans in both patients, whereas only 29% and 42% of urine glycosaminoglycans are cnondroitin sulfate. Tbe C/O ratio urine heparin sulfate of the patients is 2.32, indicating that the hexuronic acid component parts of the patient's heparin sulfate are much the same as general heparin sulfate. However, the analysis of glycosaminoglycans with DEAE-Sephadex A-25 chromatography and electrophoresis in 0.1 M HCL showed that urine heparin sulfate of the patients arc heterogeneous, and that it covers, at least, two molecular types with different amount of sulfate and charge density.

Objective To analyze the relationship between soluble brain proteoglycan (PG) and brain development. Methods SD rats were divided into three groups: new-born, young adult and near-old groups. Soluble brain PGs of each group were extracted with 4 mol?L -1 guanidine HCl and purified by DEAE-sephacel chromatography, their molecular sizes were identified by Sepharose 4B chromatography, and their GAG compositions were characterized by two dimensional electrophoresis. Results The extraction amounts of soluble brain proteoglycans were decreased with age; soluble brain PGs could be divided into four fractions according to molecule size. The percentage of the large size CSPG aggregate increased with age, but its HA declined. The percentage of the small sized fraction containing DSPG declined with age increase. Conclusion Soluble brain PGs change with age and are related to brain development.

Objective To investigate the clinical and pathological characteristics of renal involvement in Beh(c)et's disease (BD).Methods A retrospective analysis was carried out in BD patients complicated with renal damage who were admitted to Peking Union Medical College Hospital from June 1998 to July 2012.Results Twenty patients with renal involvement constituted 3.2％ of all the 618 hospitalized BD patients.The presentation of renal disease was chronic glomerulonephritis in 6 patients (1 with nephrotic syndrome),renal tubular acidosis in 1 patient,renal artery stenosis in 7 patients,renal vein thrombosis in 1 patient,and chronic renal failure of unknown etiology in 5 patients.Kidney biopsy was performed in 5 patients,3 of them revealed glomerular minor lesion,mild mesangial proliferative glomerulonephritis and chronic tubularinterstitial nephropathy,respectively.The other 2 patients underwent a second biopsy,one with glomerular minor lesion transforming into IgA nephropathy of grade Ⅲ on Lee's glomerular grading system after 6 years,and the other with IgA nephropathy of grade Ⅱ progressing to grade Ⅳ after 2 years.After the diagnosis of renal BD,one patient with uremia underwent peritoneal dialysis,while the remaining 19 patients received immunosuppressant (or with combination of glucocorticoid,angiotensin converting enzyme inhibitors and angiotensin Ⅱ receptor blockers.etc.) therapy.Among the 8 patients with renal vascular involvement,2 underwent surgery,and several received anticoagulant therapy.During the follow-up of 13 patients,the urine protein quantifications were reduced,and renal functions remained relatively stable.Conclusion Renal damage is relatively uncommon in BD patients.There are various clinical spectrums for renal BD.Routine screening with urinalysis,serum creatinine and imaging studies should be carried out for the early diagnosis of renal BD.

Background Clinical work found that triamcinolone acetonide (TA)bleeding during vitrectomy in proliferative diabetic retinopathy (PDR),but its mechanism is not clear.Objective This study was to explore the anastalsis of TA in vitrectomy for PDR.Methods A prospective study was performed.Twelve eyes of 12 patients who received vitrectomy combined with the intraocular use of TA for PDR were in cluded in Tianjin Medical University General Hospital from 2011 to 2014 and served as TA group.Thirty-two eyes of 32 patients who underwent vitrectomy for epimacular membrane or macular hole were enrolled as control group.The vitreous specimens of 0.6 ～0.8 ml was collected during the surgery.The concentrations of urokinase plasminogen activator (u-PA),tissue plasminogen activator (t-PA) and plasminogen activator inhibitors 1 (PAI-1) in vatreous were measured by ELISA.Results The mean contents u-PA,t-PA and PAI-1 in the vatreous were 25.45,127.44 and 0.42 ng/ml respectively in the TA group,and those the mean contents in the control group were 22.94,142.37 and 0.27 ng/ml respectively,shouwing a significant difference between the TA group and the control group (Z=-2.268,P＜0.05).NO significant difference was found in vitreous t-PA and PAI-1 between TA and control groups (Z =-0.092,-1.847,both at P＞0.05).Conclusions Vitreous u-PA content is increased in PDR eyes,which is more likely to lead bleeding.Anastalsis of TA during vitrectomy for PDR may be relatived to decreasing vitreous t-PA and u-PA contents as well as increasing PAI-1 contents.

Objective To analyze changes in serum levels of inflammatory factors,galectin-3 (Gal-3),fractalkine(FKN) and interleukin-6 (IL-6),in patients with dilated cardiomyopathy (DCM) or ischemic cardiomyopathy (ICM),and to explore their clinical significance.Methods Seventy-six hospitalized patients with DCM were selected to serve as a DCM group,and 78 patients with ischemic cardiomyopathy were selected into an ICM group from October 2016 to December 2017 at the Department of Cardiology,and patients in the two groups received cardiac function classification.Meanwhile,82 healthy people were included as a control group.Fasting venous blood was collected,and serum levels of Gal-3,FKN,IL-6,and BNP were measured.The correlations of Gal-3,FKN,and IL-6 with DCM or ICM were analyzed.Results Levels of plasma BNP and serum Gal-3,FKN,and IL-6 in the DCM and ICM groups were higher than those in the control group,and their levels in the DCM group were significantly higher than in the ICM group.Levels of plasma BNP and serum Gal-3,FKN,and IL-6 increased as their New York Heart Association(NYHA)classification went higher in both the DCM and ICM groups.For patients classified at the same level,serum levels of Gal-3,FKN,and IL-6 of the DCM group were significantly higher than those of the ICM group(P ＜ 0.05).BNP levels showed no difference between the two groups(P ＞0.05).Spearman correlation analysis indicated positive correlations of NYHA classification with levels of BNP(r =0.30,0.19),Gal-3(r=0.24,0.19),FKN(r =0.63,0.51),and IL-6(r =0.28,0.15)in both the DCM and ICM groups(P ＜ 0.05),and the correlation was stronger in the DCM group.Conclusions Increased expression of plasma BNP,Gal-3,FKN,and IL-6 in patients with DCM and ICM are closely related to the severity of cardiac function impairment.Monitoring changes in levels of plasma BNP and serum inflammatory factors Gal-3,FKN and IL-6 provides important clues for the differential diagnosis between DCM and ICM,the assessment of clinical conditions and treatment-related decisions.

Objective:To construct a risk prediction score for the needs of coronary care unit (CCU) care in stable condition acute ST-segment elevation myocardial infarction (STEMI) patients who receive percutaneous coronary intervention (PCI) treatment.Methods:The clinical data of 805 STEMI patients who accepted PCI in the First Hospital of Jilin University from November 2017 to October 2018 were retrospectively analyzed. Among the patients, 654 patients from November 2017 to July 2018 were served as the modeling group, the patients with needs of CCU had 125 cases, and the patients without needs of CCU had 529 cases; 151 patients from August 2018 to October 2018 were served as the validation group, the patients with needs of CCU had 28 cases, and the patients without needs of CCU had 123 cases. Binary Logistic regression analysis was used to establish the risk prediction model and determine the score standards. The critical value was determined according to the best Youden index of receiver operating characteristic (ROC) curve.Results:Among 805 patients with STEMI, 153 cases (19.01%) had the needs of CCU, and the most common reason was pump failure (heart failure and cardiogenic shock, 113 cases). In the modeling group, age (60 to 74 years old, OR = 1.513, 95% CI 0.945 to 2.424, P = 0.085; ≥75 years old, OR = 2.740, 95% CI 1.371 to 5.478, P = 0.004), total ischemic time>4 h ( OR = 1.701, 95% CI 1.022 to 2.831, P = 0.041), admission shock index ≥0.8 ( OR = 1.910, 95% CI 1.178 to 3.099, P = 0.009), multi-vessel disease ( OR = 2.090, 95% CI 1.272 to 3.432, P = 0.004), preoperative diseased vessels thrombolysis in myocardial ischemia (TIMI) blood flow grade 0 ( OR = 2.099, 95% CI 1.313 to 3.353, P = 0.002), acute anterior myocardial infarction ( OR = 3.696, 95% CI 2.347 to 5.819, P<0.001) and previous history of stroke ( OR = 3.927, 95% CI 2.057 to 7.500, P<0.001) were independent risk factors for CCU needs in STEMI patients undergoing PCI. The scoring criteria were as followings: age<60 years old was given 0 score, 60 to 74 years old 1 score, ≥75 years old 2 score; total ischemic time>4 h in 1 score, admission shock index ≥0.8 2 scores, multi-vessel disease 2 scores, preoperative diseased vessels TIMI blood flow grade 0 2 scores, acute anterior myocardial infarction 3 scores, previous history of stroke 3 scores, and the total score was 15 scores. The patients with 0 to 6 scores were low-risk, and the patients with 7 to 15 scores were high-risk. ROC curve analysis result showed that, in modeling group, the area under curve (AUC) of risk prediction score for predicting the needs of CCU in STEMI patients was 0.740 (95% CI 0.692 to 0.788, P = 0.580); in validation group, the AUC of risk prediction score for predicting the needs of CCU in STEMI patients was 0.755 (95% CI 0.658 to 0.853, P = 0.755). Conclusions:A predictive risk score based on seven risk factors such as age, total ischemic time, admission shock index, multi-vessel disease, preoperative diseased vessels TIMI blood flow grade, acute anterior myocardial infarction and previous history of stroke is constructed in order to predict the needs of CCU in STEMI patients with stable condition who receive PCI treatment. It can be used to help doctors to identify high-risk patients before the admission to CCU, thus providing simple and practical clinical tool for rational allocation of limited CCU resources.

Objective:To explore our self-designed classification system of irreducible intertrochanteric fractures based on reduction stage and bone block position and to evaluate the reduction techniques guided by the classification system.Methods:A retrospective study was conducted to analyze the data of 115 patients with irreducible intertrochanteric fracture who had been admitted to Department of Orthopedics, Beijing Hospital from September 2014 to November 2022. There were 24 males and 91 females with a mean age of (80.9±11.0) years. The reduction for the fractures was divided into a diaphysis reduction stage (Phase Ⅰ) and a cortical reduction stage (Phase Ⅱ). Based on the relative positions of the intraoperative bone blocks, Phase Ⅰ was divided into an anterior and posterior interlocking type (Phase Ⅰa) and a distal bone block sinking displacement type (Phase Ⅰb) while Phase Ⅱ into a proximal lifting type (Phase Ⅱa), a posterior angulation type (Phase Ⅱb), a positive support type (Phase Ⅱc), and a negative support type (Phase Ⅱd). Depending on the difficulties encountered in different reduction stages, corresponding close reduction strategies (such as top rod support, percutaneous prying, and Joystick technique) were adopted to restore the proximal femoral neck shaft angle, anteversion angle, anterior medial cortex, and length of the affected limb before fixation with intramedullary nails. Recorded were the patient's surgical time, intraoperative bleeding, quality of postoperative reduction, fracture union time, and complications.Results:The surgical time for this group of patients was 70.0(60.0, 92.0) minutes, and the intraoperative blood loss 200.0 (170.0, 200.0) mL. According to the standards by Baumgaertner et al., the quality of postoperative reduction was evaluated as excellent in 103 cases and as good in 12 cases, with an excellent and good rate of 100.0% (115/115). Of the 115 patients, 86 were followed up for more than 6 months to reveal fracture union in all after a duration of 6.0 (4.0, 8.0) months. One patient died of an acute cardiovascular event in the hospital 5 days after surgery. Two patients lost their mobility within 3 months after surgery due to acute cerebral infarction. There was no internal fixation failure requiring secondary surgery or no incision infection.Conclusion:Guided by our self-designed classification system of irreducible intertrochanteric fractures based on the intraoperative reduction stage and the relative position of bone block, real time intraoperative fluoroscopy images can be used to effectively clarify the difficulty of fracture reduction in stages so that corresponding reduction strategies can be adopted, leading to fine clinical efficacy.

ObjectiveTo investigate the identification of kidney Yang deficiency syndrome of patients with osteoporosis（OP）， and to form the clinical syndrome identification rules of traditional Chinese medicine（TCM）. MethodBasic information， etiology， clinical symptoms and other characteristics of 982 OP patients were included， and statistical tests were used to screen the variables associated with kidney Yang deficiency syndrome. Taking the decision tree as the base model， bootstrap aggregation algorithm（Bagging algorithm） was utilized to establish the classification model of kidney Yang deficiency syndrome in OP， generating numerous rules and removing redundancy. Combining least absolute shrinkage and selection operator（LASSO） regression to screen key rules and integrate them to construct an identification model， achieving the identification of kidney Yang deficiency syndrome in OP patients. ResultEighteen key identification rules were screened out， and of these， where 11 rules with regression coefficients>0 correlated positively with the kidney Yang deficiency syndrome， the rule with the highest coefficient was chilliness（present）&feverish sensation over the palm and sole（absent）. The other 7 rules with regression coefficients<0 correlated negatively with the syndrome， the rule with the lowest coefficient was reddish tongue（present）&diarrhea（absent）&deficiency of endowment（absent）. According to the regression coefficients of each key rule， variables with importance>0.2 were ranked as chilliness， reddish tongue， feverish sensation over the palm and sole， cold limbs， clear urine， diarrhea， deficiency of endowment， prolonged illness. The results of the partial dependence analysis of the identification model showed that compared to OP patients without chilliness， those with chilliness（present） had a 0.266 8 higher probability of being identified as having kidney Yang deficiency syndrome， indicating that this variable had the highest impact on identification of the syndrome. Similarly， compared to OP patients without reddish tongue， those with reddish tongue had a 0.141 9 lower probability of being identified as having kidney Yang deficiency syndrome， indicating that this variable had the highest impact on identifying non-kidney Yang deficiency syndrome. The accuracy， sensitivity， specificity and area under receiver operating characteristic curve（AUC） of the established kidney Yang deficiency syndrome identification model in the test set were 0.865 9， 0.853 7， 0.872 0 and 0.931 5， respectively. ConclusionA precise identification model of OP kidney Yang deficiency syndrome is conducted basing on the rule ensemble method of Bagging combining LASSO regression， and the screened key rules can explain the identification process of kidney Yang deficiency syndrome. In this research， according to the regression coefficients of rules， the importance and partial dependence of variables， combined with the thinking of TCM， the influence of patient characteristics on the identification of syndromes is described， so as to reveal the primary and secondary syndromes of identification and assist the clinical identification of kidney Yang deficiency syndrome.

This study used the Appraisal of Guidelines Research & Evaluation-Health Systems （AGREE-HS） to demonstratively compare 34 global coronavirus disease-2019 （COVID-19） health systems guidance documents （HSGs） and 6 World Health Organization （WHO） standard HSGs. The comparison involved topic， participants， methods， recommendations， and implementability， with the aim of exploring the characteristics of emergency HSGs. The results showed that the emergency HSGs had an overall average score of 49%， with topic having the highest score， recommendations having the second highest score， and participants having the lowest score. The standard HSGs had an overall average score of 79%， with high scores in all items. The emergency HSGs had lower scores in participants， methods， recommendations， and implementability than the standard HSGs （P<0.001）， while the COVID-19 emergency HSGs developed by the WHO had higher score in topic than the standard HSGs （P<0.05）. Compared with those released by countries， the COVID-19 emergency HSG developed by the WHO showed superiority in all items and overall scores （P=0.000 2）. This indicates that emergency HSGs， represented by the COVID-19 emergency HSG， place equal emphasis on topic and recommendations as standard HSGs but have low requirements in terms of expert participation， evidence support， and comprehensive consideration in the time- and resource-limited context. They have the characteristics of prominent topics， clear purposes， orientation to demand， keeping up with the latest evidence， flexible adjustment， and timeliness， emphasizing immediate implementation effects， weakening long-term effects， and focusing on comprehensive benefits. Additionally， developers， types， and report completeness are important influencing factors.