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Objective@#To investigate the clinical features, treatment strategies and long term outcomes of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.@*Methods@#The data of clinical features, auxiliary examinations, treatments and prognosis in children with anti-NMDAR encephalitis in Xiangya Hospital of Central South University from March 2014 to October 2017 were collected and retrospectively analyzed. A total of 71 patients were enrolled, including 33 males and 38 females. The youngest age of onset was 4 months old, and the age of onset was (9±4) years. The first-line immunotherapy treatment for anti-NMDAR encephalitis was short course corticosteroid (high-dose impulse therapy and oral maintenance therapy for 1 month in acute period) and (or) immunoglobulin. The clinical evaluation was performed 2 weeks after first-line immunotherapy treatment. The second-line immunotherapy treatment, including rituximab and (or) cyclophosphamide, would be started if the symptoms did not improve significantly and the modified Rankin scale (mRS) score ≥3. All patients were followed up and evaluated for prognosis. T-test, Mann-Whitney U, Chi square test and Fisher′s exact probability method were used for comparison between good outcome group and poor outcome group, first-line immunotherapy group and first-line immunotherapy combined with second-line immunotherapy group.@*Results@#The more common clinical manifestations were psychiatric symptoms (n=61, 86%), dyskinesia (n=55, 77%) and convulsions (n=51, 72%). Two cases (3%) had tumors. Electroencephalogram (EEG), cerebro-spinal fluid (CSF) and brain magnetic resonance imaging (MRI) studies were abnormal in 83% (59/71), 39% (27/69) and 38% (27/71) patients, respectively. For the treatment regimens, all the 71 patients underwent first-line immunotherapy, resulting in improvement within 14 days in 40 cases (56%), and 1 case (1%) died. The rest 30 cases (42%) received second-line immunotherapy. The patients were followed up for 5.0-41.8 months, with a median of 19.3 months. At the last follow-up, 49 cases (69%) recovered completely, 15 cases (21%) had mild disability, 6 cases (8%) had severe disability, 1 case (1%) died and 3 cases (4%) had relapse. There were significant differences between the groups with good prognosis and poor prognosis on admission to pediatric intensive care unit (PICU) and consciousness disorder (10/64 vs. 5/7, 39/64 vs. 7/7, P=0.047, 0.004). There were significant differences between first-line immunotherapy group and the first-line combined second-line immunotherapy group on admission to PICU, consciousness disorder, sleep disorder and first mRS score (12% (5/41) vs. 33% (10/30), 44% (18/41) vs. 93% (28/30), 56% (23/41) vs. 90% (27/30), 3 (1-5) vs. 4 (3-5), respectively; χ2=4.645, 18.555, 9.560, Z=5.184, P=0.031, <0.01, 0.002, <0.01, respectively).@*Conclusions@#Anti-NMDAR encephalitis can occur in all ages of children. The most common clinical manifestations are psychotic symptoms, dyskinesia and convulsions. Paraneoplastic cases are less common in children. Immunotherapy is effective. The second-line immunotherapy should be given after the failure of first-line therapy (mRS score≥3).
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Clinical data of 62 cases of tracheobronchial foreign bodies with pulmonary atelectasis admitted in Second Affiliated Hospital and Yuying Children′s Hospital during January 2007 to December 2016 were retrospectively analyzed .There were 40 boys and 22 girls aged 9 months to 10 years, and the symptom onset ranged from 6 hours to 4 months prior to medical intervention .The foreign bodies were vegetables in 55 (88.71%), pieces of meat in 3 (4.84%) and chemical product in 4 cases (6.45%). Sixty patients recovered after medical intervention , 1 died preoperatively and 1 died of severe reexpansion pulmonary edema ( RPE) .The foreign bodies were successfully removed with rigid bronchoscopy in a single attempt in 47 children (77.05%), 1 child (1.64%) required two attempts to completely remove the foreign bodies;10 children ( 16.39%) were treated with fiberoptic bronchoscopy;3 children ( 4.92%) received thoracotomy , in which 1 child ( 1.64%) received a lobectomy due to pulmonary atelectasis and lung consolidation during operation .Conclusion Foreign bodies combined with pulmonary atelectasis in children are likely to be misdiagnosed , which led to severe adverse events . RPE is a serious postoperative complication of children receiving rigid bronchoscopy as a treatment , especially those diagnosed with tracheobronchial foreign bodies combined with pulmonary atelectasis .
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AIM: To investigate the effect of hypercapnia on hypoxia-induced pulmonary hypertension and the changes of lysyl oxidase (LOX) and extracellular matrix collagen cross-links in the rat.METHODS: Sprague-Dawley rats were randomly divided into 4 groups: normoxia group, hypoxia group, hypercapnia group and hypoxia+hypercapnia group.LOX activity was detected by fluorescence spectrophotometry.LOX protein expression was detected by immunohistochemistry and Western blot.The mRNA expression of LOX in the pulmonary artery was detected by real-time PCR.RESULTS: The levels of mean pulmonary artery pressure (mPAP), RV/(LV+S) and WA/TA in hypoxia group were significantly higher than those in normoxia group (P<0.01).Moreover, the levels of mPAP and RV/(LV+S) in hypoxia+hypercapnia group were significantly lower than those in hypoxia group (P<0.01).However, no significant difference of mPAP and RV/(LV+S) between hypercapnia group and normoxia group was observed.In hypoxia group, the collagen cross-links in the lung tissue was significantly higher than that in normoxia group and hypercapnia group (P<0.01).Importantly, collagen cross-links in the lung tissue of hypoxia+hypercapnia group was significantly lower than that in hypoxia group (P<0.01).There was no significant difference in collagen cross-links between hypercapnia group and normoxia group.The expression of LOX at mRNA and protein levels and its activity in the pulmonary arteries of hypoxia group were significantly increased as compared with normoxia group (P<0.01).Furthermore, the expression of LOX at mRNA and protein levels and its activity in the pulmonary arteries in hypoxia+hypercapnia group were lower than those in hypoxia group (P<0.01).CONCLUSION: Hypoxia not only up-regulates LOX but also promotes collagen cross-linking in the rat lung, which contributes to the development of pulmonary hypertension.Hypercapnia inhibits hypoxia-induced LOX expression and collagen cross-linking, therefore impairing the progress in hypoxia-induced pulmonary hypertension.
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AIM:To explore the effect of Delta-like ligand 4 (Dll4)-Notch signaling pathway blockade on the development of Thelper 17(Th17) cells in the asthmatic mice.METHODS:Male BALB/c mice were randomly divided into 5 groups:control group, asthma group, normal saline group, anti-Dll4 antibody group, and immunoglobulin G group.The protein expression of Dll4 was detected by immunohistochemical staining.The proportion of Th17 cells in mouse spleen isolated CD4+ T cells was measured by flow cytometry.The protein expression of Th17 transcription factor retinoid-related orphan receptor γt (RORγt) was determined by Western blot.The serum level of interleukin (IL)-17 was measured by enzyme-linked immunosorbent assay (ELISA).RESULTS:The expression of Dll4 in the lung tissues from asthma group significantly increased as compared with anti-Dll4 antibody group.The proportion of Th17 cells in CD4+ T cells was significantly down-regulated, and the protein expression of RORγt in the lung tissues was significantly reduced in anti-Dll4 antibody group compared with asthma group (P<0.05).Moreover, the serum level of IL-17 in anti-Dll4 antibody group was significantly reduced compared with asthma group (P<0.01).CONCLUSION:The blockade of Dll4-Notch signaling pathway inhibits the differentiation of Th17 cells in asthmatic mice.
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Asthma is the most common chronic respiratory disease in children. Currently,inhaled corti-costeroids is the most effective drug for asthma,however,there are some adverse effect by long-time using. Ac-cording to recent literature,long-time treatment with inhaled corticosteroids may inhibit height growth. But this suppression only occurs within early stage of asthma treatment, and have no effect or slight effect on adult height. The adverse effects of inhaled corticosteroids for height are also related to its iypes,dose and usage.
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Objective To explore the clinical manifestations,imaging findings,pathological classification and treatment of congenital cystic adenomatoid malformation (CCAM)of the lung.Methods The clinical features,imaging findings,pathology information,diagnosis,treatment method and its prognosis of children with CCAMconfirmed by ope-ration and pathology were retrospectively analyzed in Yuying Children′s Hospital Affiliated to Wenzhou Medical Univer-sity from August 2006 to August 201 4.Results Eleven patients were boys and 4 patients were girls.One case had a-symptomatic clinical features,1 2 cases had pulmonary infection,1 case had recurrent chest pain,and 1 case had de-pressed deformity in sternum inferior segment.Chest CT scanning indicated that 9 cases had multiple gas cysts at unila-teral side of lung,among which 1 case was of funnel chest and pulmonary sequestration,1 case of huge cyst containing air and fluid at inferior lobe of left lung,and 4 cases of high density lung shadow;CT examination indicated that 1 case had recurrent chest pain and eventration of diaphragm of the right side combined with pulmonary sequestration.All ca-ses were treated by surgical resection,of whom 1 case was given cystectomy and sequestrectomy,diaphragmatic plication respectively,1 case complicated with funnel chest disease underwent lesion pulmonary lobectomy,sequestrectomy and minimally invasive corrective surgery in pectus excavatum (Nuss surgery),and the remaining 1 2 cases received lesion pulmonary lobectomy.All of 1 5 cases recovered well without complications.Pathological classification type of CCAMin-cluded 1 1 cases of type Ⅰ,3 cases of type Ⅱ and 1 case of type Ⅲ,among which 2 cases had pulmonary sequestration. Conclusions CCAMis a rare disease which can be discovered along with pulmonary infection.Multiple gas cysts are the most common imaging findings and the preoperative diagnosis of CCAM is mostly based on chest CT examination. Type Ⅰ and type Ⅱ are the most common pathological classification.The surgical resection should be given early surgi-cal resection and the prognosis is usually good.
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<p><b>BACKGROUND</b>Hemifacial spasm (HFS) is a facial nerve disorder characterized by episodic involuntary ipsilateral facial muscle contraction. Information on Chinese patients with HFS has not been well-characterized. This study aimed to evaluate the clinical feature and the treatment status of HFS across China.</p><p><b>METHODS</b>A cross-sectional study including 1003 primary HFS patients had been carried out in 15 movement disorder clinics in China in 2012. The investigated information was acquired from questionnaires and medical records including demographic data, site of onset, aggravating and relieving factors, treatments prior to the investigation, etc.</p><p><b>RESULTS</b>In this study, the ratio of male to female was 1.0:1.8, the mean age at onset was (46.6 ± 11.5) years. About 1.0% patients were bilaterally affected. The most often site of initial onset was the orbicularis oculi muscle. The most often affected sites were orbicularis oculi, zygomatic, and orbicularis oris muscles. Stress/anxiety and relaxation were most often aggravating and relieving factors, respectively; 2.3% patients had family history, 28.4% cases were combined with hypertension, and 1.4% patients were with trigeminal neuralgia. Botulinum toxin type A (BTX-A) injection was the most commonly used treatment, followed by acupuncture and oral medication. BTX-A maintained the highest repeat treatment ratio (68.7%), while 98.4% patients gave up acupuncture. The mean latency of BTX-A effect was (5.0 ± 4.7) days, the mean total duration of the effect was (19.5 ± 11.7) weeks, and 95.9% patients developed improvements no worse than moderate in both severity and function. The most common side effect was droopy mouth.</p><p><b>CONCLUSIONS</b>The onset age of HFS in China is earlier than that in western countries. The most often used two treatments are BTX-A injection and acupuncture, while the latter kept the poor repeat treatment ratio because of dissatisfactory therapeutic effect.</p>
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Adult , Female , Humans , Male , Middle Aged , Botulinum Toxins, Type A , Therapeutic Uses , China , Cross-Sectional Studies , Hemifacial Spasm , Diagnosis , Drug Therapy , Neuromuscular Agents , Therapeutic UsesABSTRACT
Objective To investigate the underlying causes, clinical characteristics, treatment and prognosis of non-traumatic pneumomediastinum (PM) in children. Methods A retrospective analysis of the clinical data of 64 children diagnosed with non-traumatic PM in Yuying Children’s Hospital Affiliated to Medical University from Jan 2003 to Dec 2013 was performed. Nineteen children with SPM and the other 45 with clear causes of non-traumatic PM were divided into two groups for comparison. According to age, 64 cases were divided into 0-6y group and 6-18y group for further comparison. Results A total of 64 patients with non-traumatic PM were collected. Nineteen of them aged 14.90±2.00 y had SPM with unknown etiology, and 84.2%were male. The other group of 45 patients aged 4.26±4.45y, and 55.6%of this group were male. The common causes were pneumonia or other lower respiratory tract infection, asthma and foreign body inspiration. The patients with SPM were always with chest pain. While the patients with clear causes of non-traumatic PM were more complained of dyspnea, coughing, subcutaneous emphysema. The treatment of patients with SPM was bed rest, oxygen uptaking, antitussive, anti-infection and other conservative therapy. All the patients with clear causes of non-traumatic PM had favorable prognosis with the treatment of actively curing primary disease and timely mediastinal air drainage, subcutaneous air drainage and thoracic close drainage. In 35 cases younger than 6 years old, the most common causes were pneumonia or other lower respiratory tract infection, none had SPM. In 29 cases older than 6 years, 19 of them had SPM. Conclusions Etiologies of pneumomediastinum varied with age in children,which should be vigilantly examined, especially for those younger than 6 years old. The key of the treatment to non-traumatic PM with clear etiologies was to treat its primary disease.
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Objective To explore the occurence,clinical characteristics and treatment of Mycoplasma pneumonia complicated with embolism in children.Methods Twenty-three cases with Mycoplasma pneumonia complicated with embolization were retrospectively analyzed from January 1990 to December 2012.Results The ages of cases were from 4 years old to 13 years old,and fifteen cases were male,eight cases were female.Nineteen cases with single-shot embolism included four cases of lower limb venous thrombosis,one case of internal carotid artery thrombosis,nine cases of cerebral infarction,two cases of cardiac infarction,two cases of splenic infarction,one case of pulmonary infarction; and the other four cases were multiple embolism,two cases combined pulmonary embolism and lower limb deep vein thrombosis,one case combined cardiac embolism and pulmonary embolism,one case combined internal carotid artery and the brain embolism.In addition,eight cases had temporary anti-cardiolipin antibody IgM,two cases combined protein C decrease,one case merge protein S decrease,and one case was lack of AT-Ⅲ.At last,two children died,the rest all recovered well after thrombolysis and anticoagulation therapy.Conclusion Mycoplasma pneumoniae has hypercoagulative state and potentialized to thrombosis,especially for children with high risk factors of thrombosis.Early diagnosis and anticoagulation and thrombolysis treatment actively is the key to better prognosis.
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Objective To find out more extrema simultaneously including global optimum and multiple local optima existed in multi-modal functions. Methods Germinal center is the generator and selector of high-affinity B cells, a multicellular group's artificial immune algorithm was proposed based on the germinal center reaction mechanism of natural immune systems. Main steps of the algorithm were given, including hyper-mutation, selection, memory, similarity suppression and recruitment of B cells and the convergence of it was proved. Results The algorithm has been tested to optimize various multi-modal functions, and the simulation results show that the artificial immune algorithm proposed here can find multiple extremum of these functions with lower computational cost. Conclusion The algorithm is valid and can converge on the satisfactory solution set D with probability 1 and approach to global solution and many local optimal solutions existed.
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Objective To explore the clinical characteristics of cerebral infarction accompanied with central serous chorioretinopathy (CSCP).Methods The clinical data of a patient with cerebral infarction and CSCP were reviewed retrospectively.Results The patient was a middle-aged male and mainly presented with dizzy,dystaxia on walking and dysopia.However,he had no history of hypertation,diabetes,hyperlipidemia and related vasculitis.Brain MRI scanning showed infarction in cerebellar hemisphere.Fluorescein eyeground angiography demonstrated the leakage focus on the retina of right eye.All of the presentations were obviously remissive with the treatments of improving micro-vascular circulation and nerve nourishment.Conclusion For those cases of cerebral infarction without related risk factors of cerebral stroke but presenting with dysopia,the possibility of CSCP should be considered.
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AIM: To study the effect of bone marrow stem cell transplantation on mdx mice at different ages. METHODS: The bone marrow stem cells of C57BL/6 mice (4 - to-weeks age) were cultured in vitro for 3 days, then injected intravenously into the 6 -week and 8-week aged mdx, which were preconditioned with 7 Gy ? ray. 12 weeks after being transplanted, the mdx mice were studied for the dystrophin protein expression on the skeletal muscle membrane. RESULTS: Three months after transplanted with bone marrow stem cells, about 16% and 7% muscles cells in 6-week and 8-week mdx mice expressed dystrophin protein, respectively. CONCLUSION: 12 weeks after transplantation with bone marrow stem cells of homologous series mice, different amounts of dystrophin protein expressed on the membrane of skeletal muscle cells were observed in different aged mdx mice. Bone marrow stem cell transplantation show more benefic effect for younger mdx mice.
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AIM: To detect dystrophin expression in skeletal muscles of mdx mice after bone marrow transplantation (BMT), and to evaluate the effect of BMT on Duchenne muscular dystrophy (DMD). METHODS: Bone marrow cells were cultured for three days, and then transplanted into mdx mice irradiated lethally through tail veins. After 4 and 6 months, dystrophin expression on myocytes membranes in mdx mice was detected by fluorescent immunohistochemical staining. The centrally nucleated fibers (CNF) were calculated by HE staining, and the physiologic parameters measured and the motor function detected by traction test, rotating rods test and rotating wheels test were also observed. RESULTS: Until 4 and 6 months after BMT, dystrophin was expressed partly on myocytes membranes in mdx mice, and the ratio of CNF decreased, physiologic functions improved, the motor ability reinforced in treated group. CONCLUSION: After BMT, marrow stem cells settled in injured skeletal muscles and bone marrow, then differentiated into myocytes with dystrophin expression and caused the improvement of pathology, physiology and motor function in treated group finally. These results give a powerful proof for the treatment of DMD with BMT.
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Objective Duchenne muscular dystrophy (DMD) is a lethal inherited disorder; the main pathogenesis is deficiency of dystrophin Our study is to observe dystrophin expression in myofibers of mdx mice (an animal model of DMD) transplanted with different bone marrow cells Methods Bone marrow cells, suspension cells and stromal cells were cultured in vitro from C57BL male mice, and these cells were transplanted respectively by tail vein of irradiated mdx mice Dystrophin expression of female mdx mice was detected dynamically By using PCR technique, Y chromosome specific sex determining regions were detected dynamically with the blood of transplanted female mdx mice Results Few dystrophin expression in myofibers (
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AIM: To observe skeletal muscle damage of mdx mice after overload exercise, and protection to muscle damage induced by exercise due to myoblast transplantation (MTT). METHODS: Muscle samples of C 57 mice were minced and digested with trypsin, and myoblasts were cultured ex vivo , purified and detected by immunohistochemistry stains. The myoblasts were injected into muscle of left limb of mdx mice, whereas the right limb was injected with DMEM liquid as control. Mice were submitted to exercise for 3 days starting 1 month after MTT, and then Evans blue was injected intravenously through the tail vein. The muscle cryostat sections of mdx mice were made, and then detected the immunofluorescence of dystrophin. Under a fluorescence microscope, the number of fiber stained with Evans blue and dystrophin was counted, analyzed quantitatively with image software. RESULTS: Under a fluorescence microscope, only 10 37%?2 87% muscle fibers in the myoblast grafted muscles were stained with Evans blue. In contrast, 26 82%?14 85% muscle fibers in right control muscles were stained. Significant differences between these two groups were showed ( P
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Objective To explore the clinical characteristics of adrenoleukodystrophy (ALD). Methods The clinical date of 14 ALD patients were analyzed retrospectively. Results The 14 ALD patients were boys,the symptoms occurred slowly from 1 to 13 years old. The clinical manifestations of all the ALD patients were mainly mental retardation and disturbances of movement of extremities,9 cases had visual disorders,6 cases had auditory disorders and 13 cases had dysarthria,5 cases had seizure and 6 cases had increased skin pigmentation. The level of plasma very long chain fatty acids (VLCFA) in 5 csaes were increased at some degree.The brain CT from 2 cases and MRI from 12 cases showed butterfly-like focus in periventricle areas in the white matter bilaterally. Lace-like high intensity lesions were observed in 3 cases. Conclusion The clinical features of ALD are progressive dysnoesia,limbs dyskinesia,hypropsia,hearing loss etc,dysfunction of adrenal cortex,the level of plasma VLCFA increased,and with characteristic changes of skull imageological.
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AIM: To study the role of the gene and protein expression of MIP-1? and RANTES in the bronchus of murine asthma. METHODS: 20 male BALB/C mice were randomly divided into two groups: the control group (A 0 group) and asthma group (B 0 group). In the experiment, the mice model of asthma was established by the ovalbumin (OVA) challenge methods. The protein expression of MIP-1? and RANTES were detected by immunohistochemistry methods. The gene expressions of MIP-1? and RANTES were detected by in situ hybridization methods. RESULTS: Immunohistochemistry showed that the expressions of MIP-1? protein and RANTES protein around the bronchus of group B 0 were significantly higher than those of group A 0 (P
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AIM: To investigate the correlation between the expression of neuron-specific protein and apoptosis in the process of differentiation from rat bone marrow stromal cells into neuron with brain-derived neurotrophic factor (BDNF). METHODS: The 5th passage MSCs were induced by BDNF and 2-mercaptoethanol (?-ME), respectively. At 1 h, 6 h, 12 h and 24 h, nestin, neuron specific enolase (NSE), microtubulease associated protein (MAP)-2 and glail fibrillary acidic protein (GFAP) were detected by Western blotting. Cell cycle and apoptosis were examined by flow cytometry. RESULTS: Nestin and NSE of neuron-like cells induced by BDNF and ?-ME were all positive by Western blotting. At 12 h, nestin and NSE turned to negative and apoptosis was detected in ?-ME group, nestin and NSE still positive and apoptosis wasn't detected in BDNF group. Till 24 h, nestin and NSE in BDNF group were negative but apoptosis still not detected. Notably, GFAP (glial astrocyte marker) was detected and MAP-2 wasn't detected in the two induced groups. CONCLUSION: The down-expression of neuron-specific protein correspondingly with apoptosis in the process of differentiation from MSCs into neuron with ?-ME shows that apoptosis may be one of the causes of induced cell death. BDNF induction was not the cause of apoptosis. Other factors may include for the cell death in the presence of neuron-specific protein expression induced by BDNF.
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AIM: To study the effect of signal transducer and activator of transcription 6 (STAT6) on airway inflammation of rats with asthma. METHODS: Male Sprague-Dawley rats were randomly divided into control group and asthma group. The lung tissue was sampled from the left lung. Bronchoalveolar lavage fluid (BALF) was collected from the right lung. The concentrations of IL-4 in serum and BALF were measured by sandwich ELISA. STAT6 protein and STAT6 mRNA were observed in the epithelial cells of bronchus by the technique of immunohistochemistry and in situ hybridization. RESULTS: (1) The concentrations of IL-4 in BALF and serum of asthma group were significantly higher than those in control group (P
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AIM: To observe the effects of overload exercise on skeletal muscles in X-linked muscular dystrophy(mdx) mice.METHODS: Mdx mice and C57 mice were carried out swimming and hanging tail movement tests (mdx mice as control did not exercise). It lasted for 13 minutes each time per day, and lasted 3 days. Evans blue was injected into tail vain. The mice were killed the next day, and the hind limbs were taken photographs after skins were flayed. The gastrocnemius muscles and diaphragms cryostat sections were made. Under a fluorescence microscope, Evans blue staining was seen. Then the sections were tested by routine HE staining, the histological change of muscles was analyzed under a light microscope.RESULTS: Many blue colored longitudinal lines were observed in skeletal muscles of mdx mice, whereas they were hardly seen in control mdx and C57 mice. Under a fluorescence microscope, some muscle fibers of mdx mice were stained with Evans blue, few muscle fibers of control mdx mice were stained, and C57 mice were not. Under a light microscope, HE staining of muscles showed some degenerated muscle fibers became round in shape and the myonuclei became condensed, or necrotic fibers had amorphous structures, most of them in the degenerated and necrotic fibers of diaphragms C57 mice did not have these changes.CONCLUSION: Overload exercise did harm to skeletal muscles of mdx mice; Vital staining with Evans blue is useful not only for distinguishing degenerating muscle fibers, but also for studying the degeneration process in dystrophin-deficient muscle.