Objective To study renal involvement in hepatic glycogen storage disease(GSD) in childhood. Methods One hundred and eight patients aged less than 21 years old with type Ⅰa GSD (54 cases), type Ⅲ (29 cases) and uncertain type hepatic GSD (25 cases). Urine analysis, urine albumin, urine protein of 24 h, urine ?_2-MG, BUN, creatinine, Ccr were evaluated. Results Of 108 patients with hepatic GSD, 16 patients (20.8%) had proteinuria proven by urine albumin or urine protein of 24 h, their ages first found proteinuria were 8～15 years. Two 15-year-old patients had proteinuria over 1.0g/24h. Among 72 patients, urine ?_2-MG of 51 cases (70.8%) increased (175～10 623mg/L), and the mean urine ?_2-MG of type Ⅰ a GSD was much higher than that of type Ⅲ GSD, 4138.2 and 1790.1mg/L respectively. Of 91 patients, 10 had renal insufficiency, 1/10 (15-year-old girl) had heavy proteinuria (3.5g/24h), elevated BUN (9.3mmol/L) and Scr(1061 ?mol/L). Five elder patients (11～21 years old) had hematuria with renal colic caused by renal calculus. Conclusions Persistent protenuria, increased urine ?_2-MG, decreased Ccr, and renal stones are common complications of hepatic GSD in childhood. Renal function should be thoroughly evaluated during follow-up.

Objective To investigate the expression of GFAP in rat hypothalamus after acute heat stress. Methods The rats were caged in a experimental incubator for 60 minutes,the temperature within the incubator was adjusted to 24℃,34℃,38.5℃ or 42℃,the humidity was 60%.Single anti-GFAP immunohistochemical(ABC) method and anti-Fos and GFAP double immunohistochemical method were used to observe the expression of GFAP in hypothalamus in different ambient temperatures after heat stress. Results The GFAP-positive cells were rare in hypothalamus at 24℃,however it was increased in many nuclei(anterior hypothalamic area,paraventricular nucleus,arcuate nucleus,suprachiasmatic nucleus and supraoptic nucleus)at 34℃ and peaked when ambient temperature was 38.5℃,and then decreased.However,Fos/GFAP-IR double labelled astrocytes were observed at 42℃.Conclusion Astrocytes participate in the pathophysiological process of heat stress.

Objective To report a case of progressive pseudorheumatoid dysplasia (PPD) with two kinds of WISP3 gene mutation. Methods A case of PPD was reported. Its clinical profile and the process of diagnosis were analyzed, and the related literature were reviewed. Results A 15-years old boy, who developed progressive joint pain and enlargement with spine involvement, was diagnosed as PPD. The erythrocyte sedimentation rate and C-reactive protein were in normal range, rheumatoid factor and anti-CCP antibody were all negative. HLA-B27 was also negative. Gene study discovered two kinds of mutations in Wnt1-inducible signaling pathway protein 3 (WISP3) gene: c.589+2T＞C and c.624dupA. Radiographic studies revealed severe osteoporosis without erosion, platyspondylia, enlargement of metaphysis and scoliosis deformity. The joint space of sacroiliac joint and articulation of pubis were significantly widened. Conclusion PPD is a rare autosomal recessive disorder characterized by cartilage homeostasis. It is associated with WISP3 gene mutations. Gene detection, laboratory examination and typical radiographic features are helpful for the diagnosis. This is the first report of c.589+2T＞C and c.624dupA mutations in patients with PPD in our country.

Objective To observe the effect of pre-intervention with electroacupuncture at Neiguan (PC 6) plusα2-adrenoceptor (α2AR) agonist on peri-operative heart rate variability (HRV).Method A total of 120 patients going to receive lower limb orthopedic surgery were randomized into four groups by using the random number table, namely a control group (group A), an electroacupuncture group (group C), anα2AR agonist group (group D) and an electroacupuncture plus medication group (group N), 30 cases in each group. The indicators of heart rate variability (HRV), including the standard deviation of normal-to-normal (N-N) intervals (SDNN), standard deviation of the average of N-N intervals (SDANN), root mean square successive differences (rMSSD), percentage of adjacent N-N intervals>50 ms apart (pNN50), low-frequency power (LF), high-frequency power (HF), LF/HF and total power (TP) were recorded 1 d prior and 1 d after the operation; meanwhile, the mean arterial pressure (MAP) and heart rate (HR) were recorded before incubation (T0), right after incubation (T1), 5 min after incubation (T2), right afterextubation (T3), 5 min after extubation (T4), 60 min after extubation (T5), and 180 after extubation (T6).Result In group N, the HR and MAP at the other time points were insignificantly different from those at T0 (P>0.05); in group A, the HR and MAP at T1-T6 were significantly different from those at T0 (P<0.05); in group C and D, the HR and MAP at T1-T4 were significantly different from those at T0 (P<0.05); the HR and MAP in group N were significantly lower than those in group A at T1-T6 (P<0.05) and were significantly lower than those in group C and D at T1-T4 (P<0.05); the HR and MAP in group C and D were significantly lower than those in group A at T5 and T6 (P<0.05). In group A, the LF, HF, LF/HF and TP 1 d after the operation were significantly increased compared to those 1 d prior to the operation (P<0.05); in group A and D, the SDNN, SDANN, rMSSD, and pNN501 d after the operation were significantly lower than those 1 d prior to the operation (P<0.05); the LF, HF, LF/HF, and TP in group C, D and N were significantly lower than those in group A 1 d after the operation (P<0.05); the SDNN, SDANN, rMSSD, and pNN50 in group C and N were significantly higher than those in group A and D 1 d after the operation (P<0.05). Conclusion Pre-intervention electroacupuncture plusα2AR agonist can improve the balance of cardiac sympathetic and vagus nerves, and better maintain the peri-operative hemodynamic stability.

Objective To explore the expressions and clinical values of pro-gastrin-releasing peptide(ProGRP) and carbohydrate antigen 72-4 (CA72-4) in patients with gastric cancer.Methods Ninety patients with gastric cancer and fifty healthy subjects were selected from January 2014 to December 2016 in our hospital.Serum levels of ProGRP and CA72-4 were detected by electrochemiluminescence.The relationships between ProGRP and clinicopathological characteristics,postoperative recurrence and CA72-4 were analyzed.The diagnostic values of ProGRP and CA72-4 in gastric cancer were analyzed by receiver operating characteristic (ROC) curve.Results The expressions of ProGRP and CA72-4 in patients with gastric cancer were (249.3 + 28.9) pg/ml and (148.8 + 33.5) U/ml respectively,which were significantly higher than those of healthy subjects [(14.4 ± 7.6) pg/ml and (3.8 ± 1.4) U/ml],and the differences were statistically sigificant (t =56.320,P ＜ 0.001;t =30.504,P ＜ 0.001).The expression of ProGRP in TNM stage Ⅲ-Ⅳ [(269.1 ±30.9)pg/ml] was obviously higher than that in stage Ⅰ-Ⅱ [(198.5 +23.9)pg/ml],with a significant difference (t =11.200,P ＜ 0.001).The expression of ProGRP in patients with lymph node metastasis [(259.9 ±31.4)pg/ml] was significantly higher than that in patients without lymph node metastasis [(190.3 ±26.8)pg/ml],with a significant difference (t =9.500,P ＜ 0.001).The expression of ProGRP in patients with postoperative recurrence after one year [(181.3 ± 21.7)pg/ml] was higer than that in patients without postoperative recurrence [(26.1 ± 12.8)pg/ml],with a significant difference (t =31.830,P ＜ 0.001).There was a positive correlation between serum ProGRP and CA72-4 (r =0.792,P =0.012).According to the ROC curve,the cut-off point of ProGRP was 23.6 pg/ml,and the diagnostic sensitivity was 80.0％,the specificity was 70.0％.The cut-off point of CA72-4 was 11.2 U/ml,and the diagnostic sensitivity was 60.0％,the specificity was 89.0％.The sensitivity and specificity diagnostic value of combined detection were 89.7％ and 94.8％,better than those of individual detection (x2 =6.028,P =0.009;x2 =4.675,P =0.031).Conclusion ProGRP and CA72-4 are highly expressed in the serum of gastric cancer patients,with a positive correlation.The combined detection of ProGRP and CA72-4 can improve the diagnostic sensitivity and specificity.ProGRP is significantly correlated with tumor stage,lymph node metastasis and prognosis,which may be a mew target for prevention and treatment of gastric cancer.

Objective:To investigate the clinical value of digital three-dimensional reconstruction model of pituitary adenoma in transsphenoidal endoscopic pituitary adenoma resection.Methods:Fifty-eight patients with endoscopic pituitary, admitted to our hospital from July 2020 to March 2022, were chosen. All patients accepted transsphenoidal surgery under microscope and were randomly assigned to a control group (without digital 3D reconstruction technology before surgery) and a model group (with digital 3D reconstruction technology before surgery). The tumor size, preoperative and postoperative hormone levels, surgical duration, intraoperative blood loss, extent of surgical resection, and complications 3 months after follow up were compared.Results:There was no significant difference in age, gender, tumor types, tumor sizes and Knosp grading between the two groups ( P>0.05), indicating comparability. The resection degree of pituitary tumor in model group was better than that that in control group; the proportion of patients with total and subtotal resection in the model group was significantly higher than that in the control group (24/29 vs. 16/29, P=0.045). Intraoperative blood loss, surgical duration and average hospital stay in the model group were significantly decreased as compared those in the control group ( P<0.05); the duration of tumor resection in the model group ([46.93±10.77] min) was significantly shorter than that in the control group ([56.00±12.47] min, P<0.05). The levels of prolactin and human growth hormone in the model group 1 and 7 d after surgery were decreased as compared with those in the control group, without significant differences ( P>0.05). Three months after follow-up, the incidences of diabetes insipidus, hyponatremia and hypopituitarism in model group were significantly lower than those in control group ( P<0.05). The satisfaction rate in the model group was significantly higher than that in the control group ( P<0.05). Conclusion:The construction of digital three-dimensional reconstruction model of pituitary adenoma is helpful in understanding the adjacent relationship between tumors, nerves, and blood vessels before surgery, develop personalized surgical plan and improve surgical efficiency.

Objective:To compare the efficacy of series casting with different timings after botulinum toxin injection among equinus children with spastic cerebral palsy.Methods:Sixty-three equinus children with spastic cerebral palsy were randomly divided into a control group and an experimental group. After being injected with botulinum toxin type A, 4 weeks of series casting was applied immediately in the control group, while in the experimental group it was applied 2 weeks later. Both groups were given basic rehabilitation therapy including heel walking, kicking balls, single-leg standing, standing platform training and balance training. The modified Tardieu scale, three-dimensional gait analysis, gross motor function measure 88 (GMFM-88) and a self-made questionnaire were used to evaluate muscle tone, spasticity, gait parameters, standing and walking before the treatment and 2 and 6 months afterward.Results:Immediately after the treatment there was a significant improvement in both groups′ average MTS scores (R1, R2, R1-R2 with knee extension and flexion), dorsiflexion at initial contact, peak dorsiflexion in the stance and swing phases, and dimension D (standing), and also in their average GMFM-88 (walking) scores. Two and six months later the experimental group′s average MTS scores, peak dorsiflexion in the stance phase, and dimension D (standing) score in the GMFM-88 (walking) had improved significantly compared with the control group′s averages. However, no significant differences were observed between the two groups in any of the other measurements after the treatment.Conclusion:Delaying series casting after botulinum toxin A injection can significantly reduce tone and spasticity in the plantarflexors, improve the range of motion and functioning of the ankle, and alleviate the discomfort and pain caused by series casting.

Objective:To investigate the correlation between serum cystatin C and formation of intracranial aneurysms.Methods:Patients with unruptured intracranial aneurysms hospitalized in the First People's Hospital of Kashgar from January 2016 to September 2021 were retrospectively enrolled as the case group and patients with trigeminal neuralgia in the same period as the control group. The demographic and clinical data of patients were collected, and the correlation between serum cystatin C and the occurrence of intracranial aneurysms was determined by univariate and multivariate logistic regression analysis. Results:A total of 114 patients with unruptured intracranial aneurysms and 142 patients with trigeminal neuralgia were enrolled. Univariate analysis showed that there were significant differences in triglyceride and cystatin C, as well as the proportions of male and hypertensive patients between the case group and the control group (all P<0.05). Multivariate logistic regression analysis showed that there was a significant independent negative correlation between the serum cystatin C and the risk of intracranial aneurysms (odds ratio 0.045, 95% confidence interval 0.011-0.184; P<0.001). Conclusion:Serum cystatin C may be an independent protective factor for the formation of intracranial aneurysms.

The occurrence and development of chronic insomnia involves subjective and objective factors, which can be classified as predisposing factors, precipitating factors and perpetuating factors. Cognitive behavioral therapy for insomnia (CBT-I) evolves from the pathopsychological analysis of chronic insomnia patients, and selectively combines the sleep hygiene, cognitive therapy and behavioral therapy to improve the symptoms of patients. With the popularity of internet, the digital cognitive behavioral therapy for insomnia has an convenient advantage over the traditional therapy, and has become the focus of research. Researchers have proposed multiple therapies for chronic insomnia, including drug therapy and non-drug therapy, which improve the treatment effect of chronic insomnia from different perspectives.

The clinical symptoms of chronic insomnia are various, the clinical diagnosis process needs to be combined with sleep parameters, daytime symptoms and psychological status of patients. Recently, the neuroimaging research suggests that chronic insomnia patients have certain structural changes and functional changes, which leads a new direction for the following research. The new drugs for the treatment of chronic insomnia, improving receptor specificity or increasing intervention targets, will provides more choices for patients.