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Niemann-Pick disease type C is a lipid storage disorder associated with impaired intracellular cholesterol trafficking, caused by mutations of either NPC1 or NPC2 genes.According to the age at onset of symptoms, it is divided into 5 categories, including neonatal, early-infantile, late-infantile, juvenile and adult type.There are differences in clinical manifestations and prognoses among each category.The characteristic clinical manifestations are hepatosplenomegaly, lung infiltration, vertical supranuclear gaze palsy and gelastic cataplexy.The definite diagnosis requires demonstration of unesterified cholesterol accumulated in fibroblasts cultured from skin biopsies with filipin staining and(or) of pathogenic mutation of NPC1/NPC2 genes.There is no effective treatment for this disease yet, therefore the overall prognosis is still poor.Miglustat can delay onset of the neurological symptoms, and prolong survival of partial patients.
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Objective:To investigate the effect of biological long stem hemiarthroplasty on postoperative hip joint function, serum bone markers, angiotensin Ⅱ (Ang Ⅱ) and cortisol (Cor) levels in elderly patients with intertrochanteric fractures.Methods:A total of 120 elderly patients with intertrochanteric fractures in General Hospital of Northern Anhui Coal and Power Group from June 2017 to June 2019 were selected. According to the principle of non-randomized clinical concurrent controlled study and patient′s voluntariness, they were divided into arthroplasty group and internal fixation group, with 60 cases in each group. Proximal femoral nail antirotation (PFNA) was used in the internal fixation group, and the biological long stem hemiarthroplasty was performed in the arthroplasty group. The related indexes of perioperative operation and complications, the levels of serum AngⅡ and Cor before and after operation, the levels of serum bone markers osteocalcin (OC), calcitonin (CT), alkaline phosphatase (ALP) before and after operation were compared between the two groups. After followed up for 6 months after the operation, Harris hip function score and Barthel index (BI) score, quality of life score (GQOL-74) before and after operation, and the excellent and good rate of hip joint function at 6 months after operation were compared between the two groups.Results:The time of getting out of bed in the arthroplasty group was shorter than that in the internal fixation group, the operation time was longer than that in the internal fixation group, and the amount of blood loss and postoperative drainage were higher than those in the internal fixation group, and the differences were statistically significant ( P<0.05). The postoperative complication rate in the arthroplasty group was lower than that in the internal fixation group: 8.33%(5/60) vs. 25.00%(15/60), and the difference was statistically significant ( χ2 = 6.000, P<0.05). The levels of serum AngⅡ and Cor in the two groups were higher than those before the operation at the 1st and 3rd day after the operation, but the levels of serum AngⅡ and Cor in the arthroplasty group were also higher than those in the internal fixation group: at the 1st day after the operation: (218.68 ± 42.04) mmol/L vs. (158.19 ± 34.36) mmol/L, (327.15 ± 39.08) μg/L vs. (285.42 ± 34.06) μg/L; at the 3rd day after the operation: (169.46 ± 32.73) mmol/L vs. (138.02 ± 25.97) mmol/L, (294.83 ± 33.95) μg/L vs. (262.64 ± 30.57) μg/L, and the differences were statistically significant ( P<0.05). The levels of serum OC, CT, and ALP in the two groups at 1 month and 3 months after operation were higher than those before the operation, the levels of serum OC, CT, and ALP in the arthroplasty group were higher than those in the internal fixation group: at 1 month after operation: (17.40 ± 4.25) μg/L vs. (14.96 ± 3.79) μg/L, (1.34 ± 0.49) ng/L vs. (1.15 ± 0.43) ng/L, (159.49 ± 19.75) U/L vs. (137.24 ± 17.28) U/L; at 3 months after operation: (19.18 ± 5.79) μg/L vs. (16.24 ± 4.36) μg/L, (1.46 ± 0.57) ng/L vs. (1.24 ± 0.50) ng/L, (180.94 ± 22.42) U/L vs. (163.72 ± 19.36) U/L, and the differences were statistically significant ( P<0.05). TheHarris hip function scores at 1, 3 and 6 months after the operation in the two groups were higher than those before the operation, the Harris hip function scoresin the arthroplasty group were higher than those in the internal fixation group, and the differences were statistically significant ( P<0.05). The excellent and good rate of hip joint function at 6 months after operation in the arthroplasty group was higher than that in the internal fixation group: 90.00%(54/60) vs. 75.00%(45/60), and the difference was statistically significant ( χ2 = 4.675, P<0.05). The scores of BI, GQOL-74 at 1, 3, 6 months after operation in the two groups were higher than those before operation, the scores of BI, GQOL-74 at 1, 3, 6 months after operation in the arthroplasty group were higher than those in the internal fixation group, the differences were statistically significant ( P<0.05). Conclusions:Compared with PFNA internal fixation, the treatment of elderly patients with femoral intertrochanteric fracture with biological long stem hemiarthroplasty can promote the recovery of patients, reduce complications, reduce the impact on bone markers, and more effectively improve the patient′s hip joint function, ability of daily living and quality of life, but it is more traumatic to the body and has a strong stress response.
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Objective:To investigate the etiology of pleural effusion in hospitalized children in Beijing Children′s Hospital.Methods:Clinical information of children with pleural effusion admitted to Beijing Children′s Hospital Affiliated to Capital Medical University from January 2016 to December 2018 was retrospectively analyzed.According to the etiology, the children were divided into infection group (parapneumonic pleural effusion, tuberculous pleurisy and empyema) and non infection group.According to the age, the children were further divided into ≤ 3 years old, >3-7 years old and > 7 years old groups.Classification of statistics was performed, and the etiology of pleural effusion were retrospectively analyzed.Results:Among the 1 165 children with pleural effusion, 746 cases(64.0%) were infected with pleural effusion, 697 cases (697/746, 93.4%) of who were parapneumonic effusion.In patients with parapneumonic effusion, 457 cases (61.3%) had Mycoplasma pneumonia (MP) infection.Infectious pleural effusion was more common in children >7 years old(339/479 cases, 70.8%), while non-infectious pleural effusion was prevalent in children under 3 years old(188/324 cases, 58.0%). The difference was statistically significant ( χ2=96.33, P<0.05). Among the patients with non-infectious pleural effusion, 239 cases (239/419 cases, 57.0%) had multi-system diseases and 97 cases (97/419 cases, 23.2%) had malignant pleural effusion.All the 18 deaths were non-infectious pleural effusion. Conclusions:The leading reason for pleural effusion in children is infection.The most prevalent symptom is parapneumonic effusion, which is mainly caused by MP.
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Objective@#To study the mutual effect of EPHA3 gene polymorphism, folic acid intake in early pregnancy, and the occurrence of non-syndromic cleft lip with or without cleft palate, so as to provide the oretical basis for a etiological study on non-syndromic cleft lip with or without cleft palate.@*Methods@#A total of 195 patients and 170 controls were included.The candidate site rs7650466 was genotyped by Snapshot technique.The correlation with folic acid intake in early pregnancy was analyzed.@*Result@#The rs7650466 T allele was highly associated with the pathogenesis of non-syndromic cleft lip with or without cleft palate(OR=0.2472, 95%CI=0.1567-0.3899, P=1.851×10-9). The T allele had protective effects.Folic acid intake in early pregnancy, and its interaction with EPHA3 gene, were associated with the pathogenesis of this disease(P<0.05).@*Conclusions@#EPHA3 gene, folic acid intake during the first trimester, and theinteractionsare associated with the pathogenesis of non-syndromic cleft lip with or without cleft palate.
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Objective To optimize the formulation of the sunscreen used in military training.Methods The different formulations of sunscreen were prepared according to the orthogonal design,and then applied on 3M adhesive tape posted on the color dish with a certain amount;the absorbance in 280 nm-320 nm and 320 nm-400 nm of them were measured and the arithmetic mean (A) was concluded.By comparing A,the protective effect and the main factors affecting preventive bask were determined.The preventive bask test of the optimum formula was carried out in human trials using the method of multi point increasing ultraviolet irradiation.Results OMC,TinosorbTM M,rutin,pearl powder and baicalin all had significant effects in preventing UVA.OMC had a significant effect in preventing UVB.The formula 1 (containing the five materials mentioned above) had the best effects in preventing both UVA (SPF 18) and UVB (PA+).Conclusions The formula 1 has the best effects in preventing both UVA and UVB.
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Objective To explore the correlation between TCM constitution and sleep status in escort troops. Methods Standardized TCM Constitution Questionnaire and Pittsburgh Sleep Quality Index (PSQI) were used to investigate escort troops. Factors of PSQI and constitution types were analyzed. Results The results of PSQI showed the number of escort troops who had bad sleeping (PSQI>7) was 64, which accounted for 46%of the escort troops, among which, 45 people (70.3%) were justified as biased constitution, while 19 were normal constitution (29.7%). The top three biased constitution in bad sleeping were:qi deficiency, phlegm-dampness and dampness-heat. PSQI factors such as sleep disorder, daytime dysfunction and total score had positive correlation with qi deficiency, yang deficiency, yin deficiency, dampness-heat and blood stasis, and negative correlation with normal constitution. Conclusion Sleep condition of escort troops has correlation with TCM constitution, which indicates that sleep status could be improved by regulating TCM constitution.
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Gastric cancer is the leading cause of death of patients with malignancy and the most common malignancy in China.Stomach is also a secretory organ,thus the carcinogenesis of human stomach always accompanied neuroendocrine differentiation.There is not a explicit definition of neuroendocrine differentiation so far,and the influence of which in gastric patients prognosis is in dispute.With the deep intensive research of gastric adenocarcinoma,diagnosis and treatment in gastric adenocarcinoma with neuroendocrine differentiation becomes increasingly concerned.
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The objective of this study was to prepare a biodegradable poly (DL-lactide) injectable gel of tinidazole. The formulation parameters evaluated in this study included polymer molecular weight, polymer concentration, solvent and drug loading, and orthogonal design was used to optimize the formulation. The preferable formulation was that 30% (w/w) poly(DL-lactide) (MW is 5 700) dissolved in 70% (w/w) N-methyl-2-pyrrolidone with 4%-6% (w/w) tinidazole.
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Delayed-Action Preparations , Drug Carriers , Chemistry , Gels , Lactic Acid , Chemistry , Polyesters , Polymers , Chemistry , Technology, Pharmaceutical , Methods , TinidazoleABSTRACT
Aim To investigate the anticoagulant efficacy and mechanism of a semi-synthesized sodiumβ-1,4-glucan sulfate (Na-MCS). Methods Anticoagulant activity was evaluated by means of coagulation assays in comparison with heparin. The anticoagulant mechanism of Na-MCS was disclosed by inhibitory analysis of the activities of coagulation factors using chromogenic substrates. Results 0. 6concentration. The dosage of Na-MCS required to double APTT of normal human plasma was 0.7represented a potent anticoagulation activity in vitro, which matched the efficacy of heparin in a certain range of concentrations. Na-MCS exhibited anticoagulant activity due to inhibition of the coagulation factors Ⅱa and Xa by the mediation of anti-thrombin AT-Ⅲ.