Cognitive impairment often occurs in elderly patients with heart failure.In this paper, we reviewed the possible mechanism of cognitive impairment in elderly patients with heart failure.The possible pathological mechanism of cognitive impairment in elderly patients with heart failure was discussed from the aspects of decreased cardiac output, renal damage, atrial fibrillation, inflammatory reaction, neuroregulation, depression and high homocysteine level, and the effect of cognitive impairment on heart failure was also summarized, so as to improve both the clinicians' awareness of cognitive impairment and the level of diagnosis and treatment in elderly patients with heart failure.

OBJECTIVE: To explore the clinical characteristics and genetic etiology of a patient with adolescent-onset hypomyelinated leukodystrophy with atrophy of basal ganglia and cerebellum (H-ABC). METHODS: A patient who was diagnosed with H-ABC in March 2018 at the First Affiliated Hospital of Nanjing Medical University was selected as the study subject. Clinical data was collected. Peripheral venous blood samples of the patient and his parents were collected. The patient was subjected to whole exome sequencing (WES). Candidate variant was verified by Sanger sequencing. RESULTS: The patient, a 31-year-old male, had manifested with developmental retardation, cognitive decline and abnormal gait. WES revealed that he has harbored a heterozygous c.286G>A variant of the TUBB4A gene. Sanger sequencing confirmed that neither of his parents has carried the same variant. Analysis with SIFT online software indicated the amino acid encoded by this variant is highly conserved among various species. This variant has been recorded by the Human Gene Mutation Database (HGMD) with a low population frequency. The 3D structure constructed by PyMOL software showed that the variant has a harmful effect on the structure and function of the protein. According to the guidelines formulated by the American College of Medical Genetics and Genomics (ACMG), the variant was rated as likely pathogenic. CONCLUSION The c.286G>A (p.Gly96Arg) variant of the TUBB4A gene probably underlay the hypomyelinating leukodystrophy with atrophy of basal ganglia and cerebellum in this patient. Above finding has enriched the spectrum of TUBB4A gene variants and enabled early definitive diagnosis of this disorder.
Male ; Humans ; Adolescent ; Adult ; Magnetic Resonance Imaging ; Basal Ganglia/pathology* ; Cerebellum ; Atrophy/pathology* ; Mutation ; Tubulin/genetics*

Objective:To investigate the functional imaging parameters that effectively distinguish isocitrate dehydrogenase (IDH) gene mutation status in clinical practice with long echo time (TE) point-resolved spectroscopy (PRESS) MRS.Methods:Totally 25 patients with suspected diagnosis of low grade gliomas(LGGs; Grade II) were recruited prospectively and divided into IDH mutation group and IDH wild group according to pathological results in the study. All patients were scanned with long TE PRESS MRS. In addition, IDH mutational status was determined by post-operation Sanger sequencing. The t test or Mann-Whitney U test was used to analyze the differences of 2-hydroxyglutarate (2HG), Glutamate (Glu), Glutamine (Gln) and 2HG/Glu+Gln between the IDH mutation group and the IDH wild group, then ROC curve was plotted with statistically significant indexes to obtain the efficacy of predicting IDH mutation status. Results:Of the 25 patients, 19 had IDH mutant gliomas and 6 had IDH wild-type gliomas. 2HG, Glu, Gln and 2HG/Glu+Gln in IDH mutated group were 1.42 (1.09, 1.93)mmol/L, (1.74±1.31)mmol/L, (1.68±0.66)mmol/L, 0.55 (0.28, 0.77), respectively; while the corresponding values were 0.00 (0.00, 1.30)mmol/L, (3.28±1.02)mmol/L, (2.55±1.47)mmol/L, 0.00 (0.00, 0.26) in IDH gene wild type group, respectively. The differences of 2HG, Glu, and 2HG/Glu+Gln between the two groups were statistically significant ( P values were 0.030, 0.016, 0.004, respectively). The area under the ROC curve of 2HG/Glu+Gln was the largest (0.877), and the sensitivity was the highest (84.2%). Conclusion:The integration of 2HG with Glu and Gln can effectively realize the noninvasive assessment of IDH mutation status.

Objective:To observe the urinary iodine content (UIC), breast milk iodine content (BMIC) and milk iodine excretion of lactating rats under different iodine nutrition levels, and to explore the iodine metabolism of the lactating rats under different iodine nutrition levels.Methods:Forty female Wistar rats with body weight ranging from 70 to 120 g were divided into low iodine (LI) group, normal iodine (NI) group, hight iodineⅠ (HIⅠ) group and hight iodine Ⅱ (HIⅡ) group according to body weight by random number table method, with 10 rats in each group. The rats were fed low-iodine diet, and the iodine ion concentration of drinking water in each group was 0, 325, 18 700 and 37 450 μg/L. Twenty male rats were fed according to the feed method of NI group. After 8 weeks of intervention, the male and female rats were caged and mated in a ratio of 1 ∶ 2. Milk and 24 h urine were collected on the 7th, 14th and 21st days of lactation (L7, L14 and L21), and the amount of food and drinking water consumed were recorded. The 24 h milk excretion was calculated by acute lactation test. UIC and BMIC were determined by inductively coupled plasma mass spectrometry (ICP-MS).Results:The 24 h total iodine intake of lactating rats in LI, NI, HIⅠ and HIⅡ groups were (1.84 ± 0.51), (30.51 ± 6.79), (765.95 ± 317.41) and (1 654.26 ± 560.55) μg/d, respectively. The difference between groups was statistically significant ( P < 0.001). At L7, L14 and L21, there were statistically significant differences in UIC, BMIC and milk iodine excretion at the same lactation stages among different groups ( P < 0.001). In HIⅡ group, the difference of BMIC and milk iodine excretion at different lactation stages (L7, L14, and L21) were significantly signrficant ( P < 0.05). The 24 h milk iodine excretion of LI, NI, HIⅠ and HIⅡ groups was (1.23 ± 0.85), (11.88 ± 5.23), (207.09 ± 114.51), (493.67 ± 242.47) μg, respectively. The proportion of 24 h milk iodine excretion to 24 h total iodine intake was 66.85%, 38.94%, 27.04% and 29.84%, respectively. Conclusions:About 39% of dietary iodine is supplied to offspring through milk when iodine nutrition is normal. The iodine excretion ratio of milk is increased or decreased with low and high iodine levels. These results indicate that lactating rats with different iodine nutrition levels can regulate the ratio of iodine excretion in milk through their own compensatory effect to reduce the influence of iodine deficiency and iodine excess on their offspring.

【Objective】 To form the sampling data interval by retrospectively analyzing the sampling data of quality monitoring of fresh frozen plasma, cryoprecipitates and leukocyte-free platelets in all blood stations in Hebei Province during the past 7 years. 【Methods】 The data of blood component sampling from 12 blood station quality control laboratories in Hebei from 2015 to 2021 were collected. The FⅧ content and plasma protein content of fresh frozen plasma, the FⅧ content and fibrinogen content of cryoprecipitates, and the leukocyte residuals, red blood cell mixed and platelet content of leukocyte-free platelets were taken as the objects for discrete point and fitted curve analysis. 【Results】 The FⅧ level of fresh frozen plasma: (1.36±1.1) IU/mL, 5 blood stations showed a representative overall high or low or fluctuated characteristics; Fresh frozen plasm-plasma protein items: overall mean ±SD: (61.13±16.7) g/L, four blood stations showed scattered distribution or continuous high value scattered points; Cryoprecipitates FⅧ: the overall mean ±SD: (134.25±58.7) IU/mL, four blood stations showed the differentiation characteristics of continuous high, low or stable in the middle; Cryoprecipitates-fibrinogen items: the overall mean ±SD: (215.27±83.5) mg, five blood stations showed the overall high or low and fluctuated. Leukocyte-free apheresis platelet-to-leukocyte residual items: overall mean ±SD: 0.37±0.96 (×10⁶/bag), two blood stations showed a relatively high representative overall characteristics, and the rest were concentrated between 0 and 1; The total mean ±SD of platelet-to-red blood cell mixture without leukocyte was 2.45±2.82 (×10⁹/bag), with obvious segmented concentrated distribution, and scattered distribution in 3 blood centers. Platelet content: the overall mean ±SD was 3.14±1.55 (×10¹¹/bag), many deviations were noticed in 3 blood stations, and 1 blood station showed representative overall high characteristics. 【Conclusion】 This analysis shows that the distribution status of each blood station in different items is similar. The distribution status of discrete point groups and the change trend of the concentrated part of the fitting curve show that there are some differences in the monitoring level between the quality control laboratories of each blood station, and the update of detection instruments and reagents and the selection of detection methods greatly affect the test results. The summary data presented the index interval framework formed in the past 7 years, which helped to understand the difference between the results of each laboratory, correct the accuracy of the test results, better play the guiding role of quality monitoring in the blood preparation process, and continue to enhance the standardization of the whole process of blood collection and supply in the province.

【Objective】 To conduct the laboratory quality assessment between 12 blood stations in Hebei province, analyze the results and explore the accuracy and comparability of testing, so as to improve the level of testing ability and quality management. 【Methods】 With reference to the external quality assessment rules of National Center for Clinical Laboratories and combined with the instructions of quality assessment samples, daily testing process of the laboratories were assessed. The quality indicators include blood cell count (WBC, RBC, Hb, HCT, MCV, MCH, MCHC and PLT), biochemical items (TP) and coagulation parameters (FIB and FⅧ). 【Results】 There are still problems in laboratories in terms of personnel operation, instrument maintenance and the impact of different reagent batches, especially in biochemical items and coagulation parameters. The pass rate of biochemical items was the lowest, only 72.75%, and that of blood cell count was the highest, reaching 98.75%. 【Conclusion】 With the progress of the project, the quality monitoring level of daily blood sampling tests in the quality control laboratory of each blood station has been improved. However, it is still necessary for each laboratory to improve the testing ability and quality management to a higher level in Hebei.

Objective:To evaluate the safety and efficacy of argatroban applied as alternative anticoagulant in critical illness patients underwent extracorporeal membrane oxygenation (ECMO) with contraindications of unfractionated heparin (UFH), and to further explore the effective dose of argatroban.Methods:From July 1, 2013 to February 28, 2022, there were 14 patients who admitted in the respiratory intensive care unit (RICU) of Beijing Chao-Yang Hospital received ECMO and used argatroban for anticoagulation (argatroban group). Two of them received argatroban as the initial anticoagulant. The remaining 12 patients used UFH at first, and then switched to argatroban. UFH group included 28 patients who received UFH for anticoagulation after matching the demographic characteristics. Primary endpoint was the prevalence of ECMO-related thrombotic events. Secondary endpoints included the type of thrombotic events, prevalence of ECMO-related major bleeding events, bleeding sites, ICU mortality, mortality during ECMO, liver and kidney function, thrombelastogram, blood transfusion, dosage of argatroban, the dynamic changes of coagulation variables 4 days before and 7 days after argatroban treatment.Results:In argatroban group, there were 8 patients received veno-venous ECMO (VV-ECMO), 2 patients with veno-arterial ECMO (VA-ECMO), and 4 patients with veno-arterio-venous ECMO (VAV-ECMO). In UFH group, VV-ECMO was applied in 23 patients, VA-ECMO and VAV ECMO was established in 3 patients and 2 patients, respectively. In endpoint events, the incidence of ECMO related thrombotic events in argatroban group was slightly higher than that in UFH group (28.6% vs. 21.4%). The ECMO running time in argatroban group was slightly longer than that in UFH group [days: 16 (7, 21) vs. 13 (8, 17)]. The incidence of ECMO-related bleeding events (28.6% vs. 32.1%) and mortality during ECMO (35.7% vs. 46.4%) in argatroban group were slightly lower than those in UFH group. However, the differences were not statistically significant (all P < 0.05). The platelet transfusion in argatroban group was significantly higher than that in UFH group [U: 7.7 (0, 10.0) vs. 0.8 (0, 1.0)]. The coagulation reaction time (R value) in thrombelastography in argatroban group was significantly longer than that in UFH group [minutes: 9.3 (7.2, 10.8) vs. 8.8 (6.3, 9.7)]. The maximum width value [MA value, mm: 48.4 (40.7, 57.9) vs. 52.6 (45.4, 61.5)] and blood clot generation rate [α-Angle (deg): 54.1 (45.4, 62.0) vs. 57.9 (50.2, 69.0)] in the argatroban group were significantly lower than those in the UFH group (all P < 0.05). The activated partial thromboplastin time (APTT) was prolonged after changing from UFH to argatroban in the argatroban group [seconds: 63.5 (58.4, 70.6) vs. 56.7 (53.1, 60.9)]. The PLT level showed a decreasing trend during UFH anticoagulation therapy, and gradually increased after changing to argatroban. D-dimer level was 19.1 (7.0, 28.7) mg/L after switching to argatroban, and then no longer showed an increasing trend. The level of fibrinogen (FIB) showed a decreasing trend during the anticoagulant therapy of UFH (the lowest was 23.6 g/L), and fluctuated between 16.8 and 26.2 g/L after changing to argatroban. The median initial dose of argatroban was 0.049 (0.029, 0.103) μg·kg -1·min -1, which the highest dose was in VV-ECMO patients of [0.092 (0.049, 0.165) μg·kg -1·min -1]. The initial dose of VAV-ECMO was the lowest [0.026 (0.013, 0.041) μg·kg -1·min -1], but without significant difference ( P > 0.05). The maintenance dose of argatroban was 0.033 (0.014, 0.090) μg·kg -1·min -1, VV-ECMO patients was significantly higher than those in VA-ECMO and VAV-ECMO patients [μg·kg -1·min -1: 0.102 (0.059, 0.127) vs. 0.036 (0.026, 0.060), 0.013 (0.004, 0.022), both P < 0.05]. Conclusion:Argatroban appears to be a feasible, effective and safety alternative anticoagulant for patients with contraindications to UFH who undergoing ECMO support.

【Objective】 To obtain the monitoring measurement range of quality indicators of red blood cells, plasma and derivatives and leukocyte-reduced apheresis platelets provided by blood stations in Hebei province, explore the distribution of monitoring values and the change of monitoring level, so as to further strengthen the homogenization construction of quality control laboratories in blood stations in Hebei. 【Methods】 In 2023, the sampling data of 12 blood stations in Hebei from 2015 to 2022 were collected, scatter plots were made and the range markers were set, and the "mean±SD" line was taken as the upper limit and lower limit of the measurement range. In 2024, the monitoring values in 2023 were added, and the changes of two measurement ranges were compared to analyze the stability and overall level. 【Results】 Comparison of the measurement range from 2015 to 2022 and the measurement range from 2015 to 2023 showed that the standard deviation of the content of deleukocyte suspension of red blood cells-hemoglobin, washed erythrocyte-hemoglobin, washed erythrocyte-supernatant protein, cryoprecipitate coagulation factor-FⅧ, fresh frozen plasma-FⅧ, leukocyte-reduced apheresis platelets-leukocyte residue and leukocyte-reduced apheresis platelet-red blood cell concentration decreased from 8.132 to 7.993, 6.252 to 6.104, 0.273 to 0.267, 57.506 to 56.276, 0.920 to 0.892, 0.653 to 0.644 and 2.653 to 2.603, respectively.The narrowing of the standard deviation range of the above items led to more concentrated monitoring values and reduced dispersion. Comparison of the measurement range from 2015 to 2022 and the measurement range from 2015 to 2023 showed that the mean value of leukocyte residue of the deleukocyte suspension of red blood cells, hemoglobin content of the wash erythrocyte, protein content of supernatant of the wash erythrocyte, hemolysis rate of the wash erythrocyte, FⅧ content of the cryoprecipitate coagulation factor, plasma protein content of the fresh frozen plasma, FⅧ content of the fresh frozen plasma, platelet content of the leukocyte-reduced apheresis platelets changed from 0.362 to 0.476, 44.915 to 44.861, 0.280 to 0.283, 0.137 to 0.142, 133.989 to 133.271, 60.262 to 60.208, 1.301 to 1.277 and 3.036 to 3.033, respectively, and was closer to the national standard line, which reflects an increase in the number of unqualified monitoring values or values close to the national standard line in 2023. The long-term qualified rate of coagulation items was low, and no improvement has been observed. The stability of biochemical items has been enhanced but overall deviation has occurred, with the average value close to the national standard line. The possibility of subsequent testing failure has increased. The counting items showed no obvious common characteristics. 【Conclusion】 The use of "mean±SD" in the analysis can visually display the distribution of monitoring values of different items in Hebei, forming an indicator measurement range covering the past nine years. It shows the characteristics of each item, and provides reference for subsequent quality control laboratory data analysis of each blood stations to takes active measures to improve the monitoring level.