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1.
Article in Chinese | WPRIM | ID: wpr-934573

ABSTRACT

Objective:To analyze the composition, the changes of expense structure and the influencing factors of hospitalization expenses, for reference in optimizing the cost control of day surgery.Methods:Collection of the first page data of patients with the top three diseases(varicose veins of lower limbs, chronic cholecystitis and varicocele)in the day surgery volume ranking in three tertiary general hospitals in a city in 2020. The confounding factors were eliminated through propensity matching. The structural change of hospitalization expenses was analyzed by structural change degree, and the influencing factors of hospitalization expenses were analyzed by grey correlation degree and multiple linear regression.Results:After 1∶1 propensity matching of the first page data of 752 patients with day surgery and non day surgery, 98 patients with lower extremity varicose veins, 356 patients with chronic cholecystitis and 38 patients with varicocele were finally included. Compared with non day hand, the total hospitalization cost of day surgical instruments decreased, and the cost structure changes of chronic cholecystitis, varicocele and varicose veins of lower limbs were 14.59%, 6.20% and 16.20% respectively. Among them, the general medical service fee, nursing fee and examination and laboratory fee showed a downward trend, and the fees of materials and drugs showed an upward trend. General medical service fee, nursing fee, examination and laboratory fee, clinical diagnosis fee, treatment fee, drug fee, material fee and other expenses presented a high correlation with the cost of day surgery(grey correlation>0.90). The payment method, wound healing type and discharge diagnosis can influence the cost of day surgery( P<0.05). Conclusions:Compared with non daytime surgery, the total hospitalization cost of day surgery has a certain cost control effect, but it can not reduce the cost of all projects. The main influencing factors are the internal composition of the cost, payment method and so on. The hospitals should focus on tapping the internal cost control potential of day surgery and further expanding the coverage of day surgery diseases.

2.
Article in Chinese | WPRIM | ID: wpr-933900

ABSTRACT

Objective:To investigate the effects of early-life (intrauterine and breastfeeding period) exposure to angiotensin Ⅱ type 1 receptor autoantibody (AT 1-AA) on lipid metabolism in offspring rats. Methods:Thirty-two AT 1-AA negative healthy nonpregnant specific pathogen free female Sprague Dawley rats weighing 150-170 g were randomly divided into two groups. Those in the immune group ( n=16) were subcutaneously injected with the mixture of an equal volume of Freund's adjuvant and the second extracellular loop of human-derived angiotensin Ⅱ receptor type 1 (AT1R-ECⅡ) repeatedly to establish the AT 1-AA-positive rat model by active immunization and those in the control group ( n=16) with normal saline solution. Before each immunization, blood samples were collected from the tail of rats to detect serum AT 1-AA levels of those rats in both groups, and the AT 1-AA-positive rat model was successfully established when the serum AT 1-AA was positive and its level reached a plateau. After eight weeks of immunization, the female rats in the two groups were mated with healthy AT 1-AA-negative male rats to conceive. Serum samples were collected from the maternal and offspring rats at the gestation of 18 days (G18), postnatal 21 days (P21), and from the normally fed offspring rats from the time of weaning to 12 weeks old (W12). Active immunization was not performed on the offspring throughout the experiment. The serum AT 1-AA levels of maternal and offspring rats were determined by enzyme-linked immunosorbent assay, and serum AT1-AA was positive when the ratio of AT1-AA level of the immune group over the control group ≥2.1. The blood lipid levels of maternal and offspring rats were measured by an automatic biochemical analyzer. Serum AT 1-AA levels, total cholesterol (TC), high-density lipoprotein-cholesterol [instead of high-density lipoprotein (HDL)], low-density lipoprotein-cholesterol, and free fatty acid levels of the offspring and maternal rats were determined for correlation analysis. Two independent sample t-test, linear regression analysis, and analysis of variance were adopted for statistical analysis. Results:(1) The serum levels of AT 1-AA in maternal rats at G18 and P21 in the immune group were significantly higher than those in the control group (G18: 1.170±0.190 vs 0.114±0.016, t=14.64; P21: 0.988±0.283 vs 0.084±0.006, t=9.57; both P<0.001). (2) The serum levels of AT 1-AA in the offspring at G18 and P21 in the immune group were significantly higher than those in the control group (offspring at G18: 0.948±0.220 vs 0.105±0.010, t=10.10; male offspring at P21: 0.758±0.273 vs 0.080±0.002, t=7.46; female offspring at P21: 0.774±0.274 vs 0.084±0.005, t=7.55; all P<0.001), which showed a positive correlation with those in maternal rats at the same period (offspring at G18: R=0.78; male offspring at P21: R=0.82; female offspring at P21: R=0.82; all P<0.05). However, there was no significant difference in the serum AT 1-AA level in offspring at W12 between the immune and control group ( P>0.05). (3) The serum levels of TC at G18 and P21, and HDL at P21 in maternal rats in the immune group were all higher than those in the control group [TC at G18: (2.36±0.32) vs (1.95±0.24) mmol/L, t=2.70; P21: (2.82±0.50) vs (2.18±0.26) mmol/L, t=3.41; HDL at P21: (1.94±0.33) vs (1.57±0.23) mmol/L, t=2.80; all P<0.05]. (4) Compared with the offspring in the control group, there was no significant change in lipid metabolism at G18 and W12 in the offspring in the immune group (both P>0.05). The serum levels of TC and HDL in male and female offspring at P21 in the immune group were higher than their counterparts in the control[TC in male offspring: (2.38±0.52) vs (1.83±0.30) mmol/L, t=2.73; HDL in male offspring: (1.44±0.32) vs (1.07±0.18) mmol/L, t=2.98; TC in female offspring: (2.50±0.72) vs (1.70±0.26) mmol/L, t=3.16; HDL in female offspring: (1.41±0.33) vs (1.00±0.14) mmol/L, t=3.41; all P<0.05]. (5) The serum levels of TC and HDL in male and female offspring at P21 in the immune group showed no correlation with those in maternal rats at P21 (all R<0.5, all P>0.05). The serum levels of HDL in male and female offspring at P21 in the immune group had a positive correlation with their own serum TC levels (male offspring: R=0.98; female offspring: R=0.97; both P<0.001) and also with their own serum AT 1-AA levels (male offspring: R=0.74, P=0.023; female offspring: R=0.91, P=0.001). The serum levels of TC in male and female offspring at P21 in the immune group had a positive correlation with their serum AT 1-AA levels (male offspring: R=0.72, P=0.030; female offspring: R=0.90, P=0.001). Conclusion:The early-life exposure to AT 1-AA may cause abnormal expression of TC and HDL in offspring rats.

3.
Organ Transplantation ; (6): 634-2022.
Article in Chinese | WPRIM | ID: wpr-941485

ABSTRACT

Objective To modify the mouse model of orthotopic left lung transplantation from different perspectives, aiming to establish a simpler, faster and stabler mouse model of lung transplantation. Methods Based on preliminary modified rat model of orthotopic left lung transplantation established by our team, varying extent of modifications were made regarding the tracheal intubation, cannula preparation and anastomosis procedures of orthotopic left lung transplantation in the recipient mice. Orthotopic left lung transplantation in 40 mice were performed by an operator with microsurgical experience. The dissection of the recipient's hilar structure was carried out at the plane of the hilar clamp model within the reverse-view, and the three branches (left main bronchus, pulmonary artery and pulmonary vein) of the pulmonary hilum were anastomosed in turn by the "pendulum" anastomosis method. The operation time of each procedure was recorded. The recipient mice were sacrificed at postoperative 2 weeks, and the incidence of postoperative complications was recorded. Results Lung transplantation was successfully completed in 40 mice, with no bronchial and vascular tearing or twisting, and no bleeding at the anastomosis site. The overall cardiopulmonary procurement time was (10.7±1.5) min, cannula preparation time was (16.2±1.5) min, cold ischemia time was (25.1±2.4) min, warm ischemia time was (19.4±1.6) min, and the total operation time was (57.2±2.9) min, respectively. During the follow-up from 6 to 14 days after surgery, one recipient mouse died of pleural effusion, probably caused by infection. No pneumothorax, thrombosis or atelectasis was found in the remaining recipient mice during postoperative follow-up. Conclusions The modified mouse model of orthotopic left lung transplantation based on "pendulum" anastomosis of the reverse-view plane possesses multiple advantages of short operation time, high success rate and few complications, which is expected to become an alternative model of studying pathological changes after lung transplantation and worthy of further application.

4.
Acta Pharmaceutica Sinica B ; (6): 3481-3492, 2021.
Article in English | WPRIM | ID: wpr-922809

ABSTRACT

@#Hepatocellular carcinoma (HCC) is one of the leading causes of cancer-related deaths, characterized by highly hypoxic tumor microenvironment. Hypoxia-inducible factor-1α (HIF-1α) is a major regulator involved in cellular response to changes of oxygen levels, supporting the adaptation of tumor cells to hypoxia. Bruceine D (BD) is an isolated natural quassinoid with multiple anti-cancer effects. Here, we identified BD could significantly inhibit the HIF-1α expression and its subsequently mediated HCC cell metabolism. Using biophysical proteomics approaches, we identified inhibitor of β-catenin and T-cell factor (ICAT) as the functional target of BD. By targeting ICAT, BD disrupted the interaction of β-catenin and ICAT, and promoted β-catenin degradation, which in turn induced the decrease of HIF-1α expression. Furthermore, BD could inhibit HCC cells proliferation and tumor growth in vivo, and knockdown of ICAT substantially increased resistance to BD treatment in vitro. Our data highlight the potential of BD as a modulator of β-catenin/HIF-1α axis mediated HCC metabolism.

5.
Article in Chinese | WPRIM | ID: wpr-882867

ABSTRACT

Vagus nerve stimulation is an effective treatment for drug-resistant epilepsy, and its efficacy and safety have been confirmed after over two decades of clinical practices.However, reassuring predictors to evaluate whether patients with drug-resistant epilepsy could gain benefit from vagus nerve stimulation has not been established, because the underlying mechanism remain poorly understood.Previous studies revealed that presumable patient-related predictors included age, epileptic seizure type, epilepsy etiology, epilepsy syndrome, cognitive and behavioral responsiveness, scalp electroencephalographic related signals and heart rate variability related signals.However, most of the predictors still remain controversial due to the factors such as the heterogeneity of different research design.This article aims at analyzing and concluding the information from relevant literatures, and summing up the predictors of efficacy before vagus nerve stimulation in refractory epilepsy to highlight the future work.

6.
Article in Chinese | WPRIM | ID: wpr-882810

ABSTRACT

Objective:To summarize the therapeutic effect of deep brain stimulation (DBS) for dystonia.Methods:Detailed clinical information and peripheral blood of children with dystonia at Peking University First Hospital from April 2017 to July 2020 were collected.The motor scores of Burke-Fahn-Marsden Dystonia Rating Scale were recorded of the dystonia before and after the treatment of DBS.Whole-exome sequencing was performed on children with dystonia.Then the effect of DBS was evaluated.Results:A total of 32 cases of patients with dystonia treated with DBS were enrolled, including 16 males and 16 females.Twelve cases were treated with globus pallidus internus DBS, and 20 cases were treated with subthalamic nucleus DBS.Twenty cases (62.5%) with pathogenic gene mutations were detected.Pathogenic variants in PANK2 (9 cases), KMT2B(3 cases), GNAO1 (2 cases), GCDH (2 cases), PINK1(1 case), NDUFAF6(1 case), DYT27(1 case) and ADCY5(1 case) were found.The follow-up period was 1 month to 3 years and 8 months.Only 1 case had local infection due to improper home care.The postoperative improvement was 5.66%-95.92%. Conclusions:All patients have a certain degree of relief after DBS without obvious adverse reactions.DBS is an effective treatment for pediatric dystonia.

7.
Article in Chinese | WPRIM | ID: wpr-882790

ABSTRACT

Objective:To investigate the clinical features, imaging features, treatment options and prognosis of linear scleroderma with central nervous system involvement.Methods:One case of linear scleroderma " en coup de sabre" (LSES) school-age child suffering from dizziness, vomiting and blurred vision was admitted to the Department of Pediatrics, Peking University First Hospital on March 25, 2019.The curative effect was observed after treatment.The relevant literature was searched, and the characteristics of cases and therapeutic effects were reviewed.Results:The clinical features of this case included recurrent and transient dizziness, vomiting, and blurred vision.Cranial imaging indicated abnormal signals in the left frontotemporal lobe white matter, cingulate gyrus, basal ganglia region, and corpus callosum proximal pressure part, multiple soft meningeal line enhancement and abnormal brain substance enhancement on the brain surface in the lesion area.After 2 months of combined treatment with Methotrexate(MTX) and corticosteroids, some symptoms such as dizziness and vomiting disappeared.Three months after the treatment, in the primary cerebral hemisphere and multiple calcifications in the brain parenchyma, the lesions significantly reduced in cranial imaging.The child was followed up for 11 months and displayed no clinical symptoms.New hair was dense at the alopecia area, and skin color, texture and grain were close to normal at the damaged area.In the review of domestic literature, treatment and prognosis were not involved.Foreign literatures reported 5 cases of children, with the first choice of Methylprednisolone being combined with MTX treatment, significant effect was observed, and consistent with the treatment of this case.Conclusions:In order to detect and treat them as early as possible and improve the prognosis, LSES patients should undergo cranial integrity assessment and neurological imaging examination at an early stage, regardless of clinical manifestations of nervous system involvement.

8.
Article in Chinese | WPRIM | ID: wpr-942956

ABSTRACT

Objective: To analyze the current adherence to imatinib in patients with gastrointestinal stromal tumors (GIST) in China and its influencing factors. Methods: A cross-sectional survey was conducted. Study period: from October 1, 2020 to November 31, 2020. Study subjects: GIST patients taking imatinib who were diagnosed and treated in public tertiary level A general hospitals or oncology hospitals; those who had not been pathologically diagnosed, those who never received imatinib, or those who had taken imatinib in the past but stopped afterwards were excluded. The Questionnaire Star online surgery platform was used to design a questionnaire about the adherence to adjuvant imatinib therapy of Chinese GIST patients. The link of questionnaire was sent through WeChat. The questionnaire contained basic information of patients, medication status and Morisky Medication Adherence Scale. Results: A total of 2162 questionnaires from 31 provinces, autonomous regions, and municipalities were collected, of which 2005 were valid questionnaires, with an effective rate of 92.7%. The survey subjects included 1104 males and 901 females, with a median age of 56 (22-91) years old. Working status: 609 cases (30.4%) in the work unit, 729 cases (36.4%) of retirement, 667 cases of flexible employment or unemployment (33.3%). Education level: 477 cases (23.8%) with bachelor degree or above, 658 cases (32.8%) of high school, 782 cases (39.0%) of elementary or junior high school, 88 cases (4.4%) without education. Marital status: 1789 cases (89.2%) were married, 179 cases (8.9%) divorced or widowed, 37 cases (1.8%) unmarried. Two hundred and ninety-four patients (14.7%) had metastasis when they were first diagnosed, including 203 liver metastases, 52 peritoneal metastases, and 39 other metastases. One thousand eight hundred and sixty-nine patients underwent surgical treatment, of whom 1642 (81.9%) achieved complete resection. The median time of taking imatinib was 25 (1-200) months. Common adverse reactions of imatinib included 1701 cases (84.8%) of periorbital edema, 1031 cases (51.4%) of leukopenia, 948 cases (47.3%) of fatigue, 781 cases (39.0%) of nausea and vomiting, 709 cases (35.4%) of rash, and 670 cases (33.4%) of lower extremity edema. The score of the Morisky Medication Adherence Scale showed that 392 cases (19.6%) had poor adherence, 1023 cases (51.0%) had moderate adherence, and 590 cases (29.4%) had good adherence. Univariate analysis showed that gender, age, work status, economic income, residence, education level, marriage, the duration of taking medication and adverse reactions were associated with adherence to adjuvant imatinib therapy (all P<0.05). Multivariate analysis showed that female (OR=1.264, P=0.009), non-retirement (OR=1.454, P=0.001), monthly income ≤4000 yuan (OR=1.280, P=0.036), township residents (OR=1.332, P=0.005), unmarried or divorced or widowed (OR=1.362, P=0.026), the duration of imatinib medication >36 months (OR=1.478, P<0.001) and adverse reactions (OR=1.719, P=0.048) were independent risk factors for poor adherence to adjuvant imatinib. Among patients undergoing complete resection, 324 (19.7%) had poor adherence, 836 (50.9%) had moderate adherence, and 482 (29.4%) had good adherence. Meanwhile, 55 patients with good adherence (11.4%) developed recurrence after surgery, 121 patients with moderate adherence (14.5%) developed recurrence, 61 patients with poor adherence (18.8%) developed recurrence, and the difference was statistically significant (P=0.017). Conclusions: The adherence to adjuvant therapy with imatinib in Chinese GIST patients is relatively poor. Females, non-retirement, monthly income ≤4000 yuan, township residents, unmarried or divorced or widowed, the duration of imatinib medication >36 months, and adverse reactions are independently associated with poor adherence of GIST patients. Those with poor adherence have a higher risk of recurrence after surgery. Positive interventions based on the above risk factors are advocated to improve the prognosis of patients with GIST.


Subject(s)
Aged , Aged, 80 and over , Antineoplastic Agents/therapeutic use , Chemotherapy, Adjuvant , Cross-Sectional Studies , Female , Gastrointestinal Stromal Tumors/drug therapy , Humans , Imatinib Mesylate/therapeutic use , Male , Middle Aged , Neoplasm Recurrence, Local/drug therapy
9.
Article in Chinese | WPRIM | ID: wpr-942319

ABSTRACT

Idiopathic inflammatory myopathies are a group of rare but serious diseases. The treatment of refractory idiopathic inflammatory myopathy is always challenging, especially in children. Three cases of refractory idiopathic inflammatory myopathy treated by rituximab were reported and discussed with the review of relevant literature. All were female with on-set age of 8 years and 6 months, 11 years and 7 months, 4 years and 2 months old, respectively. All had acute onset, presenting with progressive and severe muscle weakness. All lost ambulation within 1 or 2 months, with difficult swallowing and low voice. Respiratory distress occurred in case 2 after an attack of asphyxia due to an aspiration of sputum, and ventilator support was required for 1 month. Rashes were detected at the initial stage of the disease in cases 2 and 3. Patient 2 showed facial erythematous papules, spreading to her neck and hands. Patient 3 showed purplish eyelids with peri-orbital swelling, generalized edema involving all her limbs. Creatine kinase (CK) levels were markedly elevated in all the patients, ranging from 6 000 IU/L to 28 819 IU/L. Anti-SRP antibody was identified in cases 1, and anti-NXP2 antibodies were confirmed in cases 2 and 3. MRI of both thighs in all the patients showed profound muscle and fascial edema. Muscle pathology of patient 1 showed prominent fiber variation and endomysial fibrosis, with overexpression of MHC-Ⅰ. While muscle pathology in patients 2 and 3 showed scattered fiber necrosis, regeneration, endomysial edema without inflammatory cell infiltration. All the patients were diagnosed with idiopathic inflammatory myopathy and failed to the initial treatment including adequate glucocorticoids and high-dose immunoglobulin therapy. Other immunosuppressants (methotrexate, cyclophosphamide) were also tried in cases 2 and 3 with poor response. Then all the patients were treated with rituximab combined with glucocorticoids. Patient 1 regained normal strength and discontinued rituximab at the end of her last follow-up (2 years and 7 mouths). Though calcinosis developed during the follow-up period, significant improvement was noticed in cases 2 and 3 (both regained the ability to walk independently) at the end of their last follow-up after 2 years and 8 months, 3 years and 2 months respectively. Long-term rituximab therapy may improve the prognosis of refractory idiopathic inflammatory myopathy, especially with positive anti-SRP and anti-NXP2 antibodies.


Subject(s)
Child , Female , Glucocorticoids , Humans , Infant , Magnetic Resonance Imaging , Myositis/drug therapy , Rituximab
10.
Chinese Journal of School Health ; (12): 399-403, 2021.
Article in Chinese | WPRIM | ID: wpr-875707

ABSTRACT

Objective@#To test the effectiveness of psychological flexibility training on career adaptability among middle school students who undertook psychological courses based on acceptance commitment therapy and the adolescent mental flexibility model(DNA-V), and to provide a reference plan to improve the mental health of middle school students.@*Methods@#This study recruited 110 junior high school students (60 boys and 50 girls) from a middle-school in Beijing. The students were randomly divided by class into a DNA-V face-to-face course group(offline group n=33), a DNA-V online course group(online group n=40), and a regular school psychology course group(control group n=37). Louise Hayes DNA-V intervention program was condensed into a six-hour middle-school DNA-V psychology curriculum. Using the Avoidance and Fusion Questionnaire for Youth and the Career Adaptability Scale, changes in psychological flexibility and career adaptability were measured before(T1), one week after(T2), and two months after (T3) the intervention.@*Results@#Linear mixed models were used for the analysis, while controlling for demographic variables. Psychological flexibility and career adaptability in the offline group were higher at T2 and T3 than at T1(psychological flexibility t=4.22, 3.11; career adaptablity t=3.05, 4.16, P<0.01), while the difference between T2 and T3 was not statistically significant. The psychological flexibility and career adaptability of the online group were not statistically significant at T1, T2, and T3. The psychological flexibility and career adaptability of the control group increased from T1 to T2(t=4.64, 2.47, P<0.05), but T3 decreased back to a level close to T1.@*Conclusion@#In terms of both psychological flexibility and career adaptability, the DNA-V face-to-face psychology course resulted in a retention period of at least two months.

11.
Article in Chinese | WPRIM | ID: wpr-909234

ABSTRACT

Objective:To investigate the effects of sentinel lymph node biopsy utilization on operative time, intraoperative blood loss and extubation time.Methods:Sixty-two patients with breast cancer who received treatment in the First Hospital of China Medical University from January to December 2019 were included in this study. They were randomly assigned to receive either conventional breast cancer surgery (control group, n = 31) or sentinel lymph node biopsy combined with breast cancer surgery (study group, n = 31). Extubation time, operative time, intraoperative blood loss, drainage volume, breast cosmetic effect, upper limb function and complications were compared between the two groups. Results:Extubation time and operative time in the study group were (16.3 ± 1.1) hours and (61.6 ± 11.3) minutes, respectively, and they were (28.2 ± 6.4) hours and (124.2 ± 28.5) minutes, respectively in the control group. There were significant differences in extubation time and operative time between the two groups ( t = 14.922 and 16.479, both P < 0.05). Intraoperative blood loss and postoperative drainage volume in the study group were (68.7 ± 17.9) mL and (105.9 ± 19.5) mL respectively, and they were (122.4 ± 23.1) mL and (257.2 ± 36.4) mL respectively in the control group. There were significant different differences in intraoperative blood loss and postoperative drainage volume between the two groups ( t = 15.928 and 18.797, both P < 0.05). The excellent and good rate of breast cosmetic effect in the study group was significantly higher than that in the control group (93.6% vs. 83.8% , χ2 = 5.584, P < 0.05). After treatment, the difference in the upper arm circumference between the healthy and affected sides, and shoulder abduction and shoulder function score in the study group were (0.5 ± 0.1) cm, (123.7 ± 6.6) ° and (75.9 ± 4.9) points respectively, and they were (0.7 ± 0.1) cm, (120.1 ± 6.1) °, (73.5 ± 4.4) points, respectively in the control group. There were significant differences in these indices between the two groups ( t = 11.432, 12.450, 12.647, all P < 0.05). The incidence of complications in the study group was significantly lower than that in the control group (12.9% vs. 38.8%, χ2 = 6.309, P < 0.05). Conclusion:Sentinel lymph node biopsy can help improve therapeutic effects, shorten the operative time, reduce the amount of intraoperative bleeding, shorten the extubation time, and reduce the incidence of complications in breast cancer patients.

12.
Article in Chinese | WPRIM | ID: wpr-908319

ABSTRACT

Anti-myelin oligodendrocyte glycoprotein-IgG associated disorders(MOGAD) are the most common disorders among children with idiopathic central nervous system demyelination.MOGAD in children can be monophasic or multiphasic.Common clinical phenotypes include acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, and optic neuromyelitis spectrum disorders, etc.Additionally, while rare phenotypes include encephalitis, overlapping syndrome, and cranial neuritis.For children with multiphasic MOGAD, the same phenotypes mentioned above can be repeated or combined with other phenotypes.At present, the cell-based assay is recommended to detect serum MOG-IgG level as the diagnostic basis, and the persistent presence of serum MOG-IgG level is a major risk factor for recurrence.There is a lack of high-quality clinical evidence for the treatment of disease modification in multiphasic MOGAD.Alternative drugs include azathioprine, mycophenolate mofetil, rituximab, and maintenance of intravenous immunoglobulin.The prognosis depends on the clinical phenotype, course of disease, and treatment.MOGAD is being recognized, and there are still many problems needed to be solved.

13.
Article in Chinese | WPRIM | ID: wpr-908013

ABSTRACT

Objective:To analyze the clinical features and genotypes of mitochondrial complex Ⅰ deficiency due to NDUFAF5 gene mutations.Methods:Clinical data of 2 cases with mitochondrial complex Ⅰ deficiency due to NDUFAF5 gene mutations admitted in the Department of Pediatrics, Peking University First Hospital from February 2015 to July 2018 were retrospectively reviewed and followed up.Reported cases of mitochondrial complex Ⅰ deficiency due to NDUFAF5 gene mutations were searched in online databases, including the PubMed, Wanfang, Chinese Journal Full-Text Database and VIP database from January 1975 to February 2020 with " NDUFAF5" as the key word.Through literature review, clinical features and genotypes of mitochondrial complex Ⅰ deficiency due to NDUFAF5 gene mutations were summarized.Results:Case 1 showed mentor and mental regression after infection at the age of 1 year and 4 months.The condition of case 1 remained stable at the age of 5 year and 6 months at the last follow-up.Brain magnetic resonance imaging (MRI) showed multiple lesions in the white matter of the frontal and parieto-occipital lobes, basal ganglia, thalamus, cerebellum, brain stem and corpus callosum.Case 2 showed rapidly bilateral visual impairment at the age of 7 years and 4 months.The patient′s vision moderately recovered at the age of 8 years and 8 months.Brain MRI showed midbrain, periaqueductal gray, medulla oblongata and putamen lesions.Spinal MRI showed continuous lesions in the cervical cord 1-4.Genetic test showed NDUFAF5 gene c. 764C>T (p.Ala255Val) and c. 508C>T (p.Arg170Trp), homozygous c. 836T>G (p.Met279Arg) mutations in case 1 and case 2 respectively.Through online searching, 6 reports involving 14 cases were retrieved.The most common clinical phenotype was Leigh syndrome.Two cases had disease onset during the neonatal period, and their disease progressed rapidly and died within 1 year old.Eleven cases had onset during the infantile period, and 72.7% (8/11 cases) of them had a normal development.The common initial symptoms were mental or motor regression, feeding difficulty and dystonia.Seventy-two point seven percent (8/11 cases) had acute/subacute onset after infection, showing paroxysmal deterioration, and died in infancy or childhood.One patient developed dystonia in childhood and visual impairment in adulthood.Conclusions:The onset age ranged from neonatal period to childhood in patients with NDUFAF5 gene mutations, and their clinical phenotypes vary a lot.The main clinical phenotype is Leigh syndrome.Disease onset during the infantile period is frequent, and mostly presents paroxysmal deterioration after infection, while disease onset in childhood is rare.

14.
Article in Chinese | WPRIM | ID: wpr-907963

ABSTRACT

Objective:To explore the clinical manifestations and surgical outcomes of pediatric epilepsy patients with epileptic spasms (ES) as the main form of seizure, so as to analyze the correlative factors with prognosis and improve the understanding of the operation and preoperative positioning for such patients.Methods:The clinical data of patients with ES who underwent surgery therapy from June 2014 to December 2015 in Pediatric Epilepsy Center, Peking University First Hospital were collected and retrospectively analyzed.Demographic characteristics, seizure forms, etiology, electroencephalogram (EEG), cranial magnetic resonance imaging (MRI), operative methods, pathological findings as well as surgical outcomes evaluated by Engel classification during follow-up of the subjects were collected.Correlative factors with the prognosis were explored by comparing the data between patients with optimal outcome (Engel Ⅰ) and those with poor outcomes (Engel Ⅱ-Ⅳ).Results:A total of 25 pediatric patients were enrolled, including 16 males (64.0%) and 9 females (36.0%). The age of onset was (0.81±0.68) years, the age at operation was (2.98±1.63) years, and the course of disease was (2.17±1.48) years.Besides, 84.0% (21/25 cases) of the ES patients had multiple forms of seizures and partial seizure (19 cases) was the most common.MRI of the heads of all the children showed definite lesions, including 11 patients (44.0%) with lesions limited to one brain lobe and 14 patients (56.0%) involving multiple brain lobes or hemisphere.The most common etiology was focal cortical dysplasia (13 cases), followed by intracranial developmental tumors (3 cases). All patients underwent resection surgery, including resection of lesion (3 cases), single brain lobe resection (9 cases), multiple brain lobe dissection (3 cases) and hemisphere dissection (10 cases). During a follow-up period of 4.0 to 5.5 years, 1 patient was lost.Among the remaining 24 cases, 18 (75.0%) cases achieved good outcomes and wee classified as EngelⅠ, 2 cases (8.3%) and 4 cases(16.7%) were classified as Engel Ⅱand Ⅳ, respectively.The univariate comparison between the good epilepsy prognosis group and the poor epilepsy prognosis group showed that, patients whose EEG abnormalities are consistent with the anatomical lesions during the inter ictal tend to have good prognosis( P=0.006). Conclusions:(1) Optimal therapeutic effects were observed in ES patients with definite lesions treated by surgical therapy.(2) Interictal EEG consistent with the lesion side may suggest a good prognosis for surgical treatment.(3) Structural causes should be screened as soon as possible if a patient with ES is drug-refractory and presents clues of focal origin.

15.
Article in Chinese | WPRIM | ID: wpr-907909

ABSTRACT

Objective:To analyze the clinical phenotype and genetic characteristics of children with germline PIGA gene mutations. Methods:The clinical presentations, blood biochemistry, electroencephalogram (EEG), brain magnetic resonance imaging (MRI) and genetic test results of 10 children diagnosed at the Department of Pediatrics of Peking University First Hospital between January 2014 and June 2020 were analyzed.Results:All these 10 children were male, with seizures and severe developmental delay.Five out of eight cases showed hypotonia.Four out of nine cases had facial deformity or multiple organ abnormalities.The onset age of seizures ranged from one month and 28 days to 10 months, with an average age of 4.8 months.There were various types of seizures, and all patients showed focal seizures.The seizures of 6 patients in these 10 cases could be induced by fever disease.Diffuse slow waves mixed focal or multifocal discharges of interictal EEG in 9 cases with PIGA-deficient.Brain MRI showed enlarged subarachnoid space in 44.4% (4/9 cases) of patients.Slight elevated serum alkaline phosphatase could be seen in 2 cases.Genetic analysis confirmed that a total of 8 different mutation sites were found, 7 of which were unreported.In this group, 4 cases were diagnosed with multiple congenital anomalies -hypotonia -seizures syndrome 2 (MCAHS2), 5 cases were diagnosed with developmental delay and epilepsy without deformity, and one case was not classified, respectively. Conclusions:Focal seizure was common in these patients with PIGA mutations, and often induced by fever disease.Interictal EEG was characterized by diffuse slow waves mixed focal or multifocal discharges.Enlarged subarachnoid space was the most common brain MRI abnormality in these patients.The phenotype of patients only partially conformed to typical MCAHS2 manifestations, and most of them had no deformity.

16.
Article in Chinese | WPRIM | ID: wpr-905890

ABSTRACT

Objective:To observe the effect of Ginseng Radix et Rhizoma, Notoginseng Radix et Rhizoma, and Chuanxiong Rhizoma extract (GNC) on mitochondrial oxidative stress in hydrogen peroxide (H<sub>2</sub>O<sub>2</sub>)-induced aging of human umbilical vein endothelial cells (HUVECs), and explore the therapeutic mechanism of GNC on aging HUVECs. Method:The HUVECs were classified into the control group (control), H<sub>2</sub>O<sub>2</sub> model group (H<sub>2</sub>O<sub>2</sub>), H<sub>2</sub>O<sub>2</sub> + DMSO group (DMSO, 1 mL·L<sup>-1</sup>), resveratrol group (Resv, 8 μmol·L<sup>-1</sup>), and low- (200 mg·L<sup>-1</sup>), medium- (300 mg·L<sup>-1</sup>), and high-dose (400 mg·L<sup>-1</sup>) GNC (GNC-L, GNC-M, and GNC-H) groups. Except control group and H<sub>2</sub>O<sub>2</sub> group, the other groups were intervened with corresponding agents. Subsequently, 300 μmol·L<sup>-1</sup> H<sub>2</sub>O<sub>2</sub> was given to other groups except the control group for 4 h to induce aging, and then the cells were cultured in normal media for 24 h. The aging degree, cell cycle, and mitochondrial reactive oxygen species (mtROS) level were determined by SA-<italic>β</italic>-galactosidase (SA-<italic>β</italic>-Gal) staining, flow cytometry, and MitoSox red fluorescence staining, respectively. JC-10 was used as a fluorescent probe to detect the changes in mitochondrial membrane potential, and Western blot was performed to detect the expression of manganese superoxide dismutase (MnSOD) and p-p66 proteins. Result:The SA-<italic>β</italic>-gal staining results showed that H<sub>2</sub>O<sub>2</sub> group had increased blue-stained cells compared with other groups (<italic>P</italic><0.01). Compared with those in the control group, the ratio of G<sub>0</sub>/G<sub>1</sub> phase cells significantly increased (<italic>P</italic><0.05) and that of G<sub>2</sub>/M phase cells decreased (<italic>P</italic><0.05) in the H<sub>2</sub>O<sub>2</sub> group. Compared with those in the H<sub>2</sub>O<sub>2</sub> group, the proportion of G<sub>0</sub>/G<sub>1</sub> cells decreased (<italic>P</italic><0.05) while that of G<sub>2</sub>/M cells increased (<italic>P</italic><0.05) in GNC-H groups and Resv group. The fluorescence staining for determining mitochondrial ROS level showed that the H<sub>2</sub>O<sub>2</sub> group had weakened fluorescence intensity than the control, GNC-H, and GNC-M groups (<italic>P</italic><0.05). The mitochondrial membrane potential fluorescence intensity of the H<sub>2</sub>O<sub>2</sub> group was weaker than that of the control, GNC-H, GNC-M, and GNC-L groups (<italic>P</italic><0.01), as well as the Resv group (<italic>P</italic><0.05). Western blot showed that the protein level of MnSOD was significantly lower in the H<sub>2</sub>O<sub>2</sub> group than in the control, GNS-H, and GNS-M groups (<italic>P</italic><0.05), whereas the protein level of p-p66 showed an opposite trend (<italic>P</italic><0.01), indicating that the medication can alleviate the intracellular mitochondrial oxidative stress. Conclusion:GNC can delay the H<sub>2</sub>O<sub>2</sub>-induced aging of vascular endothelial cells. The GNC intervention significantly regulated the mitochondrial ROS, mitochondrial membrane potential, and related proteins MnSOD and p-p66 to alleviate oxidative stress. Chinese medicinal materials may delay the aging of vascular endothelial cells by inhibiting mitochondrial oxidative stress.

17.
Article in Chinese | WPRIM | ID: wpr-921779

ABSTRACT

This study aims to explore the effect of extract of Ginseng Radix et Rhizoma, Notoginseng Radix et Rhizoma, and Chuanxiong Rhizoma(hereinafter referred to as GNS) on the SIRT1-autophagy pathway of endothelial cell senescence induced by hydrogen peroxide(H_2O_2). To be specific, vascular endothelial cells were classified into the blank control group(control), model group(model), model + DMSO group(DMSO), resveratrol group(RESV), and GNS low-dose(GNS-L), medium-dose(GNS-M), and high-dose(GNS-H) groups. They were treated with H_2O_2 for senescence induction except the control. After intervention of cells in each group with corresponding drugs for 24 h, cell growth status was observed under an inverted microscope, and the formation of autophagosome under the transmission electron microscope. In addition, the changes of microtubule-associated protein 1 light chain 3β(LC3 B) were detected by immunofluorescence staining. The autophagy flux was tracked with the autophagy double-labeled adenovirus(mRFP-GFP-LC3) fusion protein. Dansylcadaverine(MDC) staining was employed to determine the autophagic vesicles, and Western blot the expression of sirtuin 1(SIRT1), ubiquitin-binding protein p62, and LC3Ⅱ. After H_2O_2 induction, cells demonstrated slow growth, decreased adhesion ability, raised number of SA-β-gal-stained blue ones, a certain number of autophagosomes with bilayer membrane and secondary lysosomes in the cytoplasm, and slight rise of autophagy flux level. Compared with the model group, GNS groups showed improved morphology, moderate adhesion ability, complete and smooth membrane, decreased SA-β-gal-stained blue cells, many autophagosomes, autophagic vesicles, and secondary lysosomes in the cytoplasm, increased autophagolysosomes, autophagy flux level, and fluorescence intensity of LC3 B and MDC, up-regulated expression of SIRT1 and LC3Ⅱ, and down-regulated expression of p62, suggesting the improvement of autophagy level. GNS can delay the senescence of vascular endothelial cells. After the intervention, the autophagy flux and related proteins SIRT1, LC3Ⅱand p62 changed significantly, and the autophagy level increased significantly. However, EX527 weakened the effect of Chinese medicine in delaying vascular senescence. GNS may delay the senescence of vascular endothelial cells through the SIRT1 autophagy pathway.


Subject(s)
Autophagy , Cells, Cultured , Cellular Senescence , Drugs, Chinese Herbal/pharmacology , Endothelial Cells/drug effects , Hydrogen Peroxide , Panax/chemistry , Sirtuin 1/genetics
18.
Article in Chinese | WPRIM | ID: wpr-921755

ABSTRACT

Microarray data of hippocampal tissue(HC) of the cognitively intact elderly(60-99 years old) were compared with those of the middle-aged and the young(20-59 years old) by bioinformatics techniques to initially screen out differentially expressed genes(DEGs) and then predict potential effective Chinese medicinals for the treatment of brain aging. The gene expression profile(accession: GSE11882) was downloaded from the Gene Expression Omnibus(GEO) and DEGs were screened based on R package. The key DEGs were identified by STRING, Cytoscape and the plug-in, followed by Gene Ontology(GO) term and Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway analysis. Then the key genes and the medical ontology information retrieval platform(Coremine Medical) were mapped against each other to single out the Chinese medicinals for the treatment of brain aging and construct the " Chinese medicinal-active constituent-target" network. Among the resultant 268 DEGs(246 down-regulated and 22 up-regulated), the 15 key genes were mainly involved in biological processes such as leukocyte migration, neutrophil activation, cell chemotaxis, microglia activation and response to external stimulus, and pathways such as inflammatory process, immune response, cytokine-cytokine receptor interaction, PI3 K-Akt signaling pathway, Rap1 signaling pathway, chemokine signaling pathway and Toll-like receptor signaling pathway. The potential effective Chinese medicinals were Notoginseng Radix et Rhizoma, Ginseng Radix et Rhizoma, Salviae Miltiorrhizae Radix et Rhizoma and Astragali Radix. The analysis of DEGs and key genes enhances the understanding of the mechanisms of brain aging. This study provides potential gene targets and ideas for the development of Chinese medicine for brain aging.


Subject(s)
Adult , Aged , Aged, 80 and over , Brain , China , Computational Biology , Gene Expression Profiling , Gene Ontology , Humans , Middle Aged , Transcriptome , Young Adult
19.
Article in Chinese | WPRIM | ID: wpr-864127

ABSTRACT

Objective:To summarize the abnormalities in brain magnetic resonance imaging (MRI) and clinical manifestations of children with infantile spasms (IS) in the course of Vigabatrin (VGB) treatment.Methods:The imaging features of children with IS who took VGB orally and presented with brain MRI changes in Peking University First Hospital between September 2016 and June 2018 were analyzed retrospectively.Their use of VGB and the imaging findings were followed up.Meanwhile, the imaging and clinical features of 83 cases described in literature were summarized.Results:(1) Ten children diagnosed as IS were included.The average duration of VGB treatment at the time of imaging changes was 4.1 months, the average age was 11.8 months, and the average maximum dose was 90.6 mg/(kg·d). Brain MRI showed hyperintensities in bilateral thalamus, brainstem, basal ganglia and dentate nucleus diffusion-weight imaging (DWI), with or without T2WI, and T2 fluid attenuated inversion recovery(FLAIR) or slight hyperintensities.Brain MRI repeated in 5 patients 7-12 months later revealed that the original abnormal signals completely disappeared.Among them, 4 patients stopped using VGB and 1 patient continued to take VGB.(2) Literature review: 83 cases with IS treated with VGB from 16 literatures were reviewed, and the incidence of abnormal brain MRI was 22%-32%.The average age at initial VGB treatment was 8.0 months, and the average dose of VGB was 157.1 mg/(kg·d) when the brain MRI abnormalities were found.The MRI imaging showed high DWI signals in bilateral symmetrical thalamus, brainstem, basal ganglia (mainly pallidum) and dentate nucleus.During the follow-up of the 41 cases, no imaging abnormality was observed in 36 cases, improvement in 4 cases, and no significant change in 1 case.When MRI abnormalities were identified, 12.0%(10/83 cases) of the patients presented new clinical symptoms mainly in the extrapyramidal system, and the clinical symptoms of all children disappeared during the follow-up.Conclusions:During the course of VGB treatment of infantile spasms, brain MRI may suggest hyperintensities of DWI in the thalamus, brainstem, basal ganglia and dentate nucleus, but most are reversible.

20.
Article in Chinese | WPRIM | ID: wpr-873198

ABSTRACT

Objective:To discuss the clinical efficacy of modified Zuoguiwan on postmenopausal osteoarthritis of knee (KOA) with deficiency of liver and kidney based on theory of syndrome differentiation and treatment of kidney, and its effect on endocrine hormone and cartilage metabolism.Method:One hundred and forty patients of KOA were randomly divided into control group (70 cases) and observation group (70 cases) by random number table. Patients in two group got glucosamine hydrochloride capsule for 12 weeks, 1 capsule/time, 2 times/day, and those with obvious pain was added celecoxib capsules for 4 weeks, 0.2 g/time, 1 time/day. The control group took Kang Zengsheng pills orally. patients in observation group was also added with modified Zuoguiwan for 12 weeks, 1 dose/day. Before and after treatment, pain level during activity and rest by visual simulation of pain (VAS) were scored. And visual scale of osteoarthritis index (WOMAC) of Western Ontario and McMaster University, knee osteoarthritis severity index (ISOA), deficiency of liver and kidney, self-rating anxiety scale (SAS) and self-rating depression scale (SDS) were scored. And levels of serum estradiol (E2), follicle stimulating hormone (FSH), luteinizing hormone (LH), transforming growth factor-β (TGF-β), interleukin-1β (IL-1β), matrix metalloproteinase-3 (MMP-3) and tumor necrosis factor-α (TNF-α) were scored.Result:Scores of VAS during activity and rest in observation group were lower than those in control group (P<0.01). The total score of WOMAC, scores of pain, stiffness, joint function, deficiency of liver and kidney, SAS and SDS were all lower than those in control group (P<0.01). Total score of ISOA, symptoms and signs, maximum walking distance, daily life were lower than those in control group (P<0.01). Levels of E2, FSH, LH, TNF-α, IL-1β and MMP-3 were lower than those in control group (P<0.01). Level of TGF-β was higher than that in control group (P<0.01). And total clinical effective rate in observation group was 89.23%(58/65), which was higher than 74.60%(47/63) in control group (χ2=4.793, P<0.05).Conclusion:Modified Zuoguiwan can obviously alleviate symptoms, improve joint function, regulate the level of endocrine hormone, relieve anxiety and depression, regulate the environment of cartilage metabolism, inhibit inflammatory reaction, and improve the patients' ability of daily life and clinical efficacy.

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