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1.
Article in Chinese | WPRIM | ID: wpr-909341

ABSTRACT

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.

2.
Article in Chinese | WPRIM | ID: wpr-620004

ABSTRACT

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.

3.
Article in Chinese | WPRIM | ID: wpr-510041

ABSTRACT

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.

4.
Article in Chinese | WPRIM | ID: wpr-510040

ABSTRACT

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.

5.
Article in Chinese | WPRIM | ID: wpr-498215

ABSTRACT

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.

6.
Article in Chinese | WPRIM | ID: wpr-496408

ABSTRACT

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.

7.
Article in Chinese | WPRIM | ID: wpr-496361

ABSTRACT

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.

8.
Article in Chinese | WPRIM | ID: wpr-488178

ABSTRACT

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.

9.
Article in Chinese | WPRIM | ID: wpr-488174

ABSTRACT

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.

10.
Article in Chinese | WPRIM | ID: wpr-476974

ABSTRACT

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.

11.
Article in Chinese | WPRIM | ID: wpr-460432

ABSTRACT

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.

12.
Article in Chinese | WPRIM | ID: wpr-457571

ABSTRACT

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.

13.
Article in Chinese | WPRIM | ID: wpr-448587

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.

14.
Article in Chinese | WPRIM | ID: wpr-448300

ABSTRACT

Objective To observe the effect of GPR30 agonist G1 on high glucose-induced endoplasmic reticulum stress ( ERS) in endothelial EA .hy926 cells.Methods EA.hy926 endothelial cells were divided into three groups:nor-mal control group (Con, 17.51 mmol /L glucose), high glucose (HG, 33.3 mmol /L), high glucose +G1 group (HG+G1, HG +1 μmol/L G1).The apoptosis rate of endothelial cells was measured by flow cytometry , the protein expres-sion changes of ERS related molecules Bip , IRE1, PERK and apoptotic molecules Bax , Bcl-2 were measured by Western blot, the mRNA expressions of Bip and CHOP were measured by RT-PCR assay.Results Compared with Con group , the apoptosis in HG group was significantly increased (P <0.01), Bip, IRE1, PERK and apoptotic molecule Bax were upreg-ulateded (P <0.05, P<0.01 or P <0.001), Bcl-2 downregulatted (P <0.01) and Bip mRNA, CHOP mRNA expres-sion were upregulated (P <0.001 and P<0.01).Compared with the HG group, apoptosis rate in HG +G1 group was significantly lower (P <0.05), BIP, IRE1, PERK and apoptotic molecules Ba.0 downregulated ( P <0.05 or P <0.01), Bcl-2 expressions was increased (P <0.05), Bip mRNA and CHOP mRNA expression were decreased (P<0.001 or P<0.01).Conclusion GPR30 agonist G-1 inhibits EA.hy926 ERS in endothelial cells.

15.
Article in Chinese | WPRIM | ID: wpr-452708

ABSTRACT

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 .

16.
Article in Chinese | WPRIM | ID: wpr-435584

ABSTRACT

Objective To investigate the clinical features of spinal muscular atrophy typeⅡ. Methods The data of clinical manifesta-tion, laboratory data, onset, diagnosis, and rehabilitation of 53 outpatients suffering from spinal muscular atrophy typeⅡ(SMA-Ⅱ) were analyzed. Results Among 53 patients with SMA-Ⅱconfirmed by molecular genetic tests, 27 patients were male while 26 were female. The mean age was 3.35 years (ranged 0.75~7.8 years), and the age receiving gene diagnosis was 17.27 months. Only 15%had a family history, and abnormalities were found in 23%patients' mothers during pregnancy. 83.4%of them had water choke cough, while 87%expectoration weakness. None of them had cough assist machines and had regular spirometry monitoring. 53%of the patients took semi-liquid diet, how-ever, none of them used stomach tube. Symmetrical flaccid paralysis was so remarkable, even muscle strength of lower limbs in 87.7%of these patients were only grades 1-2. 92%had scoliosis, while 83%had tendon contracture. EMG showed extensive neurogenic changes. All children did not accept normal pre-school education, and 85%patients did not accept formal rehabilitation. Conclusion Most of phenotype of SMA-Ⅱfor the children was similar and more severe in China. Most of the patient didn't get formal education, rehabilitation and care.

17.
Chinese Journal of Neurology ; (12): 624-627, 2008.
Article in Chinese | WPRIM | ID: wpr-398551

ABSTRACT

Objective To determine whether rizatriptan has an effect on cortical spreading depression (CSD) and c-Fos expression within periaqueductal grey (PAG) induced by CSD in rats. Methods The experimental SD rats were randomly divided into group A injected with KCl, group B KCl plus rizatriptan and group C NaCL The number and amplitude of CSD were recorded after KCl or NaCl injection. C-Fos positive neurons of different layer were identified by the immunohistochemical technique 2 hours after the first injection of KCl or NaCl. Results There was no CSD in group C. The number of CSD in group A ( 10.70±3.23 ) was significantly more than that in group B (6.10±2.56, t = - 3.528, P < 0.01 ). The amplitude of CSD in group A ( 17.33 (95% CI 11.45--23.11 ) mV) was significantly greater than that in group B (11.82 (95%CI 9.24--14.70) mV, Z= -4.360, P< 0.01). There were more cFos-like immnoreactive neurons in every layer in group A than in group C (P < 0.01 ) and in group B (P < 0.05 ). Conclusion Rizatriptan has an inhibitory effect on CSD, which might induce the headache through exciting the neurons in PAG.

18.
Article in Chinese | WPRIM | ID: wpr-567674

ABSTRACT

Headache is a common complaint in the elderly.After the age of 65,more than 13% of women and 7% of men have complaint of headache.Although tension headache and migraine are the most common primary headache,we should also recognize that their clinical features are atypical,and we must consider more serious disorders in every elderly patient presenting with a headache.Secondary headache syndromes are also more common in older people.One of these,temporal arteritis,needs to be recognized and promptly treated with corticosteroids to avoid permanent visual loss.Other causes of secondary headache that are common in older people include subdural hematomas,trigeminal neuralgia,herpes zoster infection,malignancies and medicine,etc.So we should pay more attention to the headache in the elderly,and any headache occurring for the first time in someone over the age of 65,or a changing headache pattern in a patient with chronic headaches,necessitates a complete evaluation to look for an underlying cause.

19.
Article in Chinese | WPRIM | ID: wpr-541056

ABSTRACT

Objective To study the clinical manifestations and pathological features of distal myopathies, we investigated 71 distal myopathy patients collected in the past 16 years.Methods Clinical manifestations and pathological features of biopsied muscle specimens were summarized and analysed retrospectively. Results Twenty-six of the 71 patients were of Nonaka type, 38 of Miyoshi type, 2 of tibial muscular dystrophy (TMD) type, 4 of Welander type, and 1 of oculophayngodistal myopathy (OPDM) type. Nonaka type is a sporadic or autosomal recessively inherited disorder with preferential involvement of the anterior tibial muscles. In the 26 patients with Nonaka myopathy, the onset age ranged from 8 to 39 years, averaging 24 years, and the disease was rather rapidly progressive. Sternocleidomastoid, biceps brachii muscle, pectoralis major muscle and quadriceps femoris were also involved as the disease advanced. The serum CK was slightly elevated or normal. Muscle biopsies showed rimmed vacuoles markedly without obvious dystrophic features. 15-20-nm cytoplasmic and nuclear filaments were usually seen on electron microscope. The patients with Miyoshi type were characterized clinically by sporadic or autosomal recessive inheritance, preferential gastrocnemius muscle involvement, and dystrophic muscle pathology. Rimmed vacuoles were occasionally seen. Average age of onset was 23 years (range of 8-41 years). As the disease advanced, patients with Miyoshi myopathy occasionally showed apparent proximal muscle involvement. Serum CK was markedly elevated, ranging from 3-180 times than the normal. Welander type was found in four cases. Onset age was from 30 to 46 years. Weakness always began in the finger and wrist extensors. As the disease progressed, symptoms were spreading to the distal lower extremities slowly. The serum CK level was normal or only slightly elevated. Muscle biopsies showed dystrophic features, with rimmed vacuoles occasionally. The oneset ages of two patients with TMD were from 41 to 42 years. Weakness was confined mainly to the anterior tibial muscle. Muscle biopsies revealed fibre necrosis and regeneration. Rimmed vacuoles were present significantly. OPDM was found in a 38-year-old-onset patient with autosomal dominant inheritance and characterized by the weakness of distal lower extremity and development of extraocular muscles, vocal cord and pharyngeal muscle weakness. Muscle biopsies showed rimmed vacuoles without fibre necrosis.Conclusion Five types of distal myopathies were present in China, and Miyoshi and Nonaka myopathies were more common. The clinical and pathological findings of Chinese distal myopthies should be basically similar to those reported by other countries.

20.
Article in Chinese | WPRIM | ID: wpr-594573

ABSTRACT

Objective To study the clinical and pathological features of myofibrillar myopathy.Methods Clinical and pathological data of a patient with myofibrillar myopathy were anlysised retrospectively.Results The patient showed middle-aged onset,progressively proximal muscle weakness,mildly general muscle atrophy.Neurogenic changers were found in lower extremities on EMG.On mucle biopsy,cytoplasmic bodies presented in many muscle fibers in which disorganized myofibrillar networks and abnormal desmin aggregates were found,and rimmed vacuoles appeared in muscle fibers.Conclusions There is no specificity of clinical manifestation in myofibrillar myopathy.Cytoplasmic bodies,disorganized myofibrillar networks and abnormal desmin aggregates are the distinctively pathological features in myofibrillar myopathy.

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