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Duchenne muscular dystrophy (DMD) is a fatal X-linked genetic disease caused by complete absence of Dystrophin due to mutation of DMD gene. Typical symptoms are progressive muscle loss which cause slow walking, gait abnormalities, falls, and difficult squatting. The adult patients often died from the respiratory and cardiovasucula failure in their early life stage.The early introduction of steroids has altered the natural history of the disease, but can exacerbate weight gain in a population already susceptible to obesity. Prior to commencing steroids, anticipatory guidance for weight management should be provided. Malnutrition is a feature of end stage disease requiring a multidisciplinary approach, such as texture modification and supplemental feeding. Micronutri- ent requirements are yet to be determined but, as a result of corticosteroid treatment, vitamin D and calcium should be supplemented. The multidisciplinary management of boys with DMD including nutrition treatment has progressed significantly and improved the life qulity of both patients and their parents.
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Objective To analysis the expression levels of inflammatory cytokines in patients with cluster headache during headache attack period and intermittent period using antibody chips to explore the role of inflammatory cytokines in the pathogenesis of cluster headache. Methods Blood samples from 6 patients with cluster headache were collected during headache attack period and intermittent period. Samples were then centrifugated and stored at - 80 degrees refrigerator. Samples were further labeled with biotin and reacted with antibody chips against 40 major inflammatory cytokines. The target proteins were conjugated with streptomycin antibody labeled with infrared fluorescent agent, and signals were transformed to images by Licor-odyssey scanner. Results In pairwise comparisons, the levels of some inflammatory cytokines were significantly increased during attacks compared to intermittent period including interleukin-1β(44.18 vs. 68.46, P<0.05), interleukin-6(23.08 vs. 36.40, P<0.05), interleukin-8(151.87 vs. 328.12, P<0.05), interleukin-13(23.93 vs. 38.87, P<0.05), monoeyte chemoattraetant protein (454.80 vs. 725.75, P<0.05) and macrophage inflammatory protein-1β (265.08 vs. 515.74, P<0.05). Conclusion Inflammatory cytokines may play an important role in the pathogenesis of cluster headache. However, the mechanism needs further investigation.
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Duchenne muscular dystrophy is the most prevalent muscular dystrophy caused by dystrophin gene mutations. In the central nervous systerm, there are at least six dystrophin isoforms, in which Dp140 is closely associated with cognitive impairment. This paper re-viewed the discovery, structure, gene location, detection methods and function of Dp140.
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Dystrophinopathies are a group of fatal X chromosome recessive genetic disease. With the application of ventilator technolo-gy, the main causes of death in patients with dystrophinopathies turned from respiratory failure into circulatory failure, and the importance of heart problems has become increasingly prominent. Myocardial damage can be defined with myocardial enzymes, electrocardiogram, ul-trasound cardiogram, cardiac magnetic resonance imaging, etc. Drugs for chronic heart failure remain the most important strategy, and heart transplant or left ventricular assist devices implant may be the alternatives for those in the end.
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Targeting the problem that it is difficult for traditional data management mode to effectively manage TCM information, this article analyzed from the aspect of object characteristics of TCM information resources and summarized that the reason lies in the TCM information resources with big data 4V features, proposed strategies for TCM information resource management based on big data technology, and expounded the feasibility.
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Objective To observe the pyramidal neurons in the hippocampal CA1 area varying with time after heat stroke in rats. Meth-ods Sprague-Dawley rats were divided into sham group (n=5) and heat stroke group (n=14), and the heat stroke group was divided into 7-day subgroup and 21-day subgroup (n=7 at each group) after heat stroke. Heat stroke group was established model of heat stroke. The brain tissues of rats were observed with Nissl staining to count the living pyramidal neurons in hippocampal CA1 area. Results The number of living pyramidal neurons in hippocampal CA1 area decreased in the heat stroke group (F=11.80, P<0.01), and decreased more in the 21-day subgroup than in the 7-day subgroup (P<0.05). Conclusion Pyramidal neurons in hippocampal CA1 area decrease with time after heat stroke, which may be associated with the learning and memory impairment.
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Objective To investigate the cognitive condition in patients with Duchenne muscular dystrophy (DMD). Methods Wechsler Intelligence Scale for Children IV (WISC-IV) was administered for 127 DMD patients aged 6~14 years from April, 2013 to December, 2015. The verbal intelligence quotient (VIQ), performance intelligence quotient (PIQ) and full-scale intelligence quotient (FSIQ) scores were analyzed. Results The FSIQ, VIQ and PIQ were (93.2±18.6), (91.5±18.9), and (96.6±19.5), respectively, which were lower than 100 in normal population (P<0.001), and higher than that of patients abroad in the previous studies (P<0.001). There were 13 patients (10.24%) whose scores of FSIQ were less than 70. The VIQ-PIQ discrepancy score was (-5.0 ± 16.6). Conclusion About 1/10 of Chinese DMD pa-tients impaire in cognitive function, which is significantly lower than that abroad. VIQ is more affected than PIQ.
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Objective To compare the spatial learning and memory function of heat stroke rats in different periods, to explore the long-term impairment. Methods 42 Sprague-Dawley rats were randomly divided into heat stroke 7 days group (HS7, n=21), heat stroke 21 days group (HS21, n=21), and another 18 rats were performed femoral artery intubation as surgery control group (sham, n=18). They were tested with Morris water maze 7 days and 21 days after modeling respectively for 5 days. The escaping latency, the frequency of crossing the platform area and the duration in the target quadrant were recorded. Results Compared with the sham group, the escaping latency prolonged in HS7 group in all the time (P0.05). Conclusion The impairment of spatial learning and memory is the most seriously 7 days after heat stroke in rats, and it may remain for long time.
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High-resolution magnetic resonance imaging (HRMRI) is a safe, non-invasive, inexpensive, accurate and reproducible clini-cal imaging modality, and the results can acutely reflect the extent of atherosclerotic disease and have high predictive values for future clini-cal events. It uses black blood imaging techniques and can obtain sufficient sensitivity for submillimetre imaging. HRMRI has been used widely to visualize the vessel wall and differentiate the components of atherosclerotic plaques. It is of important clinical meaning to evaluate plaque vulnerability with HRMRI, which is related to the remodeling pattern, plaque burden, intraplaque hemorrhage and contrast enhance-ment, and plaque distribution, etc.
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@#Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disorder, and the carriers of DMD can suffer from cardiac damage without muscle weakness, which can predispose to death. Consequently detecting and preventing cardiac damage in the early stage can improve the carriers' quality of life. The detection of early cardiac abnormalities and the evaluation of cardiac function for carriers mainly rely on the different examination methods, including electrocardiogram, echocardiogram, cardiovascular magnetic resonance, radioactive nuclide myocardial perfusion imaging and coronary angiography. Carriers of DMD with cardiac damage are assessed by different examination methods in different ways.
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@#Objective To observe the learning and memory function of rats after heat stroke. Methods 60 Sprague-Dawley rats were divided randomly into heat stroke group (n=44), sham group (n=8) and control group (n=8). They were tested with the Morris Water Maze 7 days after modeling. The escaping latency was recorded in the first 5 days, and it was recorded with frequency crossing the platform area and the duration in the target quadrant after ridding the platform on the 6th day. Results 16 rats died in the heat stroke group after modeling. The escaping latency increased (P<0.05), and the cumulative duration in the target quadrant and the frequency of crossing the previous platform decreased (P<0.05) in the heat stroke group compared with the other groups. Conclusion The learning and memory ability is impaired after heat stroke in rats.
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@#Objective To investigate the sniff nasal inspiratory pressure (SNIP) of Chinese boys. Methods 299 healthy boys aged 5-12 years were measured with SNIP. Their height, weight, and age were recorded. Results The SNIP averaged (76.9±22.6) cmH2O, and was (59.1±14.2) cmH2O, (76.9±19.5) cmH2O, (85.3±23.5) cmH2O and (83.0±22.6) cmH2O in the boys of 5-6 years old, 7-8 years old, 9-10 years old and 11-12 years old, respectively. It was significantly different among boys of 5-6 years old, 7-8 years old and 9-10 years old (P<0.05), but no significant difference between 9-10 years old and 11-12 years old (P=0.55). Regression analysis indicated significant relations of SNIP with age, height, weight and Body Mass Index (P<0.01). Conclusion The SNIP of Chinese healthy boys are different from abroad. It is necessary to establish the Chinese children SNIP reference range.
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Duchenne muscular dystrophy (DMD) is an X-linked recessive neuromuscular disorder, and the carriers of DMD can suffer from cardiac damage without muscle weakness, which can predispose to death. Consequently detecting and preventing cardiac damage in the early stage can improve the carriers' quality of life. The detection of early cardiac abnormalities and the evaluation of cardiac function for carriers mainly rely on the different examination methods, including electrocardiogram, echocardiogram, cardiovascular magnetic reso-nance, radioactive nuclide myocardial perfusion imaging and coronary angiography. Carriers of DMD with cardiac damage are assessed by different examination methods in different ways.
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Objective To observe the learning and memory function of rats after heat stroke. Methods 60 Sprague-Dawley rats were di-vided randomly into heat stroke group (n=44), sham group (n=8) and control group (n=8). They were tested with the Morris Water Maze 7 days after modeling. The escaping latency was recorded in the first 5 days, and it was recorded with frequency crossing the platform area and the duration in the target quadrant after ridding the platform on the 6th day. Results 16 rats died in the heat stroke group after modeling. The escaping latency increased (P<0.05), and the cumulative duration in the target quadrant and the frequency of crossing the previous plat-form decreased (P<0.05) in the heat stroke group compared with the other groups. Conclusion The learning and memory ability is impaired after heat stroke in rats.
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Objective To investigate the sniff nasal inspiratory pressure (SNIP) of Chinese boys. Methods 299 healthy boys aged 5-12 years were measured with SNIP. Their height, weight, and age were recorded. Results The SNIP averaged (76.9 ± 22.6) cmH2O, and was (59.1±14.2) cmH2O, (76.9±19.5) cmH2O, (85.3±23.5) cmH2O and (83.0±22.6) cmH2O in the boys of 5-6 years old, 7-8 years old, 9-10 years old and 11-12 years old, respectively. It was significantly different among boys of 5-6 years old, 7-8 years old and 9-10 years old (P<0.05), but no significant difference between 9-10 years old and 11-12 years old (P=0.55). Regression analysis indicated significant relations of SNIP with age, height, weight and Body Mass Index (P<0.01). Conclusion The SNIP of Chinese healthy boys are different from abroad. It is necessary to establish the Chinese children SNIP reference range.
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@# Duchenne's muscular dystrophy (DMD) is a common X-linked recessive neuromuscular disorder that associated with a spectrum of genetically based cognitive and behavioral disabilities. Several mechanisms have been discussed as the cause of cognitive impairment in patients with DMD: absence of different dystrophin isoforms, impairments in neurogenesis, changes of synaptic plasticity, blood-brain barrier alterations, brain metabolic differences, cerebellum disfunction, etc.
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@#The discovery of microRNAs (miRNAs) in mammalian cells has renewed the focus on posttranscriptional regulatory mechanisms during pathogenesis. The studies have showed that miRNAs play a key role in atherosclerosis development and progress, and were reviewed in this paper.
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@#Objective To investigate the sniff nasal inspiratory pressure (SNIP) of Chinese boys. Methods 299 healthy boys aged 5-12 years were measured with SNIP. Their height, weight, and age were recorded. Results The SNIP averaged (76.9±22.6) cmH2O, and was (59.1±14.2) cmH2O, (76.9±19.5) cmH2O, (85.3±23.5) cmH2O and (83.0±22.6) cmH2O in the boys of 5-6 years old, 7-8 years old, 9-10 years old and 11-12 years old, respectively. It was significantly different among boys of 5-6 years old, 7-8 years old and 9-10 years old (P<0.05), but no significant difference between 9-10 years old and 11-12 years old (P=0.55). Regression analysis indicated significant relations of SNIP with age, height, weight and Body Mass Index (P<0.01). Conclusion The SNIP of Chinese healthy boys are different from abroad. It is necessary to establish the Chinese children SNIP reference range.
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Objective To define the functional mechanisms of two NSAIDs , paracetamol and ibuprofen , in specif-ic brain regions in headache control by observing the distribution of Fos -immunoreactive neurons in trigeminal ganglia and trigeminal nucleus caudalis in conscious rat models of vasculogenic headache .Methods Thirty male Sprague-Dawley rats were randomly divided into three groups: control group ( saline group ) , acetaminophen group and ibuprofen group .Each rat was given electrical stimulation ( frequency 20 Hz, current 3-5 mA, pulse width 0.25 ms) at 50 minutes after injec-tion .The rats were killed and perfusion fixed after electrical stimulation .Trigeminal ganglia and trigeminal nucleus caudalis of the brains were taken out for paraffin sections and immunohistochemical staining , and Fos-immunoreactive neurons were counted under the Image J system .Results After electrical stimulation , there were significant differences of Fos protein expression in bilateral trigeminal ganglia and spinal trigeminal nucleus caudalis between the saline group and groups of non -steroidal anti-inflammatory drugs , but no significant difference of Fos protein expression in bilateral trigeminal ganglia and spinal trigeminal nucleus caudalis between the acetaminophen group and ibuprofen group .Conclusions The changes of Fos expression in bilateral trigeminal ganglia and spinal trigeminal nucleus caudalis after treatment with NSAIDs suggest that such structures participate in the pain transmission and expression and the pharmacology course of analgesic drugs .
ABSTRACT
Duchenne's muscular dystrophy (DMD) is a common X-linked recessive neuromuscular disorder that associated with a spec-trum of genetically based cognitive and behavioral disabilities. Several mechanisms have been discussed as the cause of cognitive impair-ment in patients with DMD: absence of different dystrophin isoforms, impairments in neurogenesis, changes of synaptic plasticity, blood-brain barrier alterations, brain metabolic differences, cerebellum disfunction, etc.