Objective To investigate the reference intervals of serum kalium ion (K+), natrium ion (Na+), chlorine ion (Cl-), total calcium (TCa), total carbon dioxide (TCO2) and fasting blood glucose (FBG) among healthy children and adolescents(aged from 3 months to 18 years) by dry chemical system in Shenzhen.Methods Healthy children and adolescents were divided into four groups by age according to the rule of growth and development: 3 months to 3 years, -6 years, -12 years and -18 years age groups. The serum was from the remaining specimens of routine physical examination after routine testing. Serum K+, Na+, Cl-, TCa, TCO2 and FBG were detected by the VITROS 350 system, and the differences between different age groups and different genders were compared and the reference intervals of 6 items among healthy children and adolescents aged from 3 months to 18 years were established.Results A total of 1 898 qualified specimens from children and adolescents were recruited, including 1 033 males and 865 females, with 163, 791, 613 and 331 subjects for 3 months to 3 years group, -6 years group, -12 years group and -18 years group. ① TCa in boys was significantly lower than that in girls, but FBG of -6 years group in boys was significantly higher than that in girls, there was no significant difference between genders in other groups (P>0.05). Na+, Cl-,TCa and FBG significantly varied among age groups in each gender (P<0.05), however K+and TCO2 did not (P>0.05). ②Reference intervals of serum K+, Na+, Cl-, TCa, TCO2 and FBG were 3.4-4.9, 134-147, 98-110, 2.18-2.60, 19-28, 3.6-6.0 mmol·L-1 respectively. ③The overall detection rate of IFG was 2.3% (2.9% for male and 1.6% for female).There was no detectale IFG in 3 months to 3 years group,the detection rate of IFG in -6 years, -12 years, -18 years groups was 1.0%, 3.4% and 4.5%, respectively, varying significantly among age groups (P<0.05), being more common in boys than that in girls (P<0.05).Conclusion Reference intervals of serum K+, Na+, Cl-, TCa, TCO2 and FBG are established by dry chemical system among healthy children and adolescents in Shenzhen. It has little difference in different ages, genders and previous studies of domestic and foreign.
Objective The aim of this study was to investigate the use of continuous veno-venous hemodiafiltration (CVVH) in infants with acute renal failure after cardiac surgery.MethodsTwelve infants who received CVVH after cardiac surgery for congenital heart disease from Jan. 2013 to Aug. 2014 were retrospectively studied.Results All infants weighed less than 10 kg. Eleven infants were applied CVVH when peritoneal dialysis (PD) was ineffective and one infant had to rely on CVVH because of peritonitis. The mean age at receiving CVVH was 8.8±6.5 months (ranging from 1 month to 24 months) and the mean body weight was 6.6±1.9 kg (ranging from 3.5 to 9.5 kg). The median duration of renal replacement therapy was 25 hours. Serum and urine creatinine concentration were (17.8±8.1) mmol·L-1 and (169.64±38.24) μmol·L-1 before, and (5.5±2.2) mmol·L-1 and (81.4±25.1) μmol·L-1 after CVVH (Ps≤0.001), respectively. The concentration of platelet was decreased after CVVH (P=0.001). The mean artery pressure and vasoactive-inotropic score did not significantly varied before and after CVVH ［mean artery pressure: (59.0±16.2) mmHg vs. (55.2±12.4) mmHg, P=0.273; vasoactive-inotropic score: (20.3±8.5) vs. (19.9±12.0), P=0.878］. Seven infants died in the postoperative period, and the mortality rate was 58.3%. Five infants died from MODS caused by low cardiac output syndrome, one infant died from severe pulmonary infection, and one infant died from MODS caused by peritonitis. Compared with the survivors, vasoactive-inotropic score of non-survivors was significantly higher (median 27.5 vs. 15.0, P=0.017), while the mean arterial pressure was significantly lower ［(48.0±10.2) vs. (65.2±7.2), P=0.009］.Conclusion CVVH is an alternative method of renal support to improve renal function for critically ill infants after cardiac surgery. Vasoactive-inotropic score and mean arterial pressure before CVVH treatment may be the important factors influencing the prognosis of patients. CVVH treatment may lead to thrombocytopenia.
Objective To analyze the associated factors of refractory Mycoplasma pneumoniae (Mp) pneumonia.Methods The cases of Mono-Mp pneumonia admitted in Children′s Hospital of Fudan University from September 2012 to August 2013 were collected, and divided into two groups, refractory Mp pneumonia(RMPP) and usual Mp pneumonia(UMPP) . Laboratory outcomes collected by literature review were performed on the next day of admission. Chest X-ray and/or CT data were also collected in the first 3 days after admission. The laboratory outcomes and chest images were analyzed for the associated factors of refractory Mp pneumonia by univariate and multivariate logistic regression analysis.Results 653 cases of Mono-Mp pneumonia were collected, covered 51.7% (653/1257) of the total number of pneumonia. There were 300 cases in refractory pneumonia group, 171 boys and 129 girls. And there were 353 cases in normal pneumonia group, 221 boys and 132 girls. ①Univariate analysis showed the age in RMPP group was significantly higher than that of UMPP group, (66.8 ± 37.5) vs (51.4 ± 34.4) months, P<0.01; the proportion of cases over 3 years old in RMPP group was also significantly higher than that in UMPP group (234/300 vs 224/353, P<0.01); the hospitalized days and febrile days in RMPP group were significantly higher than those in UMPP group (P<0.01, respectively); laboratory outcomes in RMPP group, CK, LDH, HBDH, ALT, AST, CRP, PCT, IL-6, were significantly higher than those in UMPP group (P<0.01, respectively); infiltration area of chest images in RMPP was significantly greater than that in UMPP, (1.95 ± 1.12) vs (1.55 ± 0.97), P<0.01. ② Those significant outcomes in univariate analysis were chosen to perform stepwise logistic regression, and the analysis showed that febrile days (OR=1.954,95%CI:1.403-2.722), serum LDH level (OR=1.009,95%CI:1.001-1.018), and infiltration area of chest images （OR=2.422,95%CI:1.111-5.279） were the independent associated factors of RMPP. Conclusion Mp became a major pathogen of hospitalized children with CAP. Severe cases often occurred in children over 3 years old. The persistent fever, the large area of infiltration in chest images, high level of serum LDH in the early stage suggested that it may progress into severe pneumonia.
Objective To systematically evaluate the effectiveness and safety of beta-receptor blocker(β-R) for infantile hemangiomas treatment.Methods Online databases of Cochrane library, PubMed, EMBASE, Web of Science Chinese biomedical literature database (CBM), CNKI and VIP were comprehensively searched for relevant randomized controlled trials (RCTs). Quality assessment was conducted by 6 items proposed by Cochrance handbook and data were analyzed by using RevMan 5.3 software.Results A total of 17 RCTs with 1 097 patients were included (treatment group: 585 cases, control group: 512 cases). Meta-analysis result showed that propranolol was better than prednisone(OR=6.15, 95%CI: 1.69-22.40), worse than laser(OR=0.13, 95%CI: 0.02-0.67), while had no significant difference with timolol(OR=0.64, 95%CI: 0.31-1.35). The efficacy of timolol did not differ from propranolo（OR=1.80,95%CI:0.14-23.37,P=0.65） and higher than that in no treatment (OR=21.80, 95%CI:7.10-66.90, P<0.05).β-R also had advantages in aspects of onset time, tumor shape and color changing time. For safety, β-R did not cause additional complications of thyroid, liver and kidney, in which propranolol had less complications than prednisone(OR=0.20, 95%CI: 0.06~0.65), while more complications than timolol(OR=5.74, 95%CI: 2.30-14.36). Heart rate, hepatic function and renal function were not obviously influenced during β-R therapy. Conclusion β-R significantly improved clinical treatment outcomes for infantile hemangiomas, and without serious adverse events. Among different kinds of drugs, timolol had an advantage of administration mode, meanwhile to some extent decreased the rate of complications, while this finding needed to be supported by further studies.
Objective To estimate the effect of nebulized magnesium sulfate on treating children with acute asthma.Methods Electronic literature search was performed up to July 2014 at the Cochrane Library, PubMed, EMBASE, CBM, CNKI, VIP and Wanfang database. The randomized controlled trails(RCTs) of nebulised magnesium sulphate for treating acute asthma in children were obtained, including the use of nebulized MgSO4 combined with bronchodilators and compared with the use of bronchodilators. The pulmonary function and adverse drug reaction were selected as primary and secondary outcomes. Data were pooled using random effects meta-analysis of the standardized mean difference (SMD) in pulmonary function by RevMan software. The subgroup analysis was performed for each pulmonary function parameter and magnesium sulfate nebulized with and without other bronchodilators.Results A total of 8 RCTs involving 1 161 patients were included. ①Nebulized MgSO4 had no significant effect upon respiratory function(10 min：SMD=-0.32，95%CI:-0.67 to 0.04，P=0.08；20 min：SMD=-0.11，95%CI:-0.40 to 0.17，P=0.43) in children. ②Subgroup analysis:MgSO4 was significantly inferior to salbutamol upon pulmonary function when compared with salbutamol (10 min：SMD=-0.75，95%CI:-1.29 to -0.22，P=0.005；20 min：SMD=-0.46，95%CI -0.72 to -0.20,P=0.006); MgSO4 didn't show significant effect upon respiratory function(FEV1 %predicted, PEF %predicted) to saline when used in combination with bronchodilators(FEV1% predicted after 10 min：SMD=-0.03，95%CI:-0.52 to -0.46，P=0.89；PEF% predicted after 10 min：SMD=-0.02，95%CI:-0.23 to 0.19，P=0.83；FEV1% predicted after 20 min：SMD=0.04，95%CI: -0.29 to 0.37，P=0.81; PEF% predicted after 20 min: SMD=0.05，95%CI: -0.31 to 0.42，P=0.78). ③Common adverse drug reaction of MgSO4, such as arrhythmia, decrement of myotasis reflex were not reported in included 8 studies. There was no difference in incidence of adverse drug reaction between MgSO4 and control groups.Conclusion The use of nebulized MgSO4 in the treatment of acute asthma appears to be less benefit upon improving pulmonary function in children when compared with salbutamol, and to be without significant effect upon improving pulmonary function compared with salbutamol.
Objective To systematically review the efficacy and safety of intermittent use of diazepam to prevent recurrence of febrile seizure (FS) using meta-analysis methods.Methods The Cochrane Library (Issue 7, 2014), PubMed, EMBASE, CBM, CNKI, VIP and Wanfang Data were searched for the randomized controlled trails (RCTs) about prophylactic use of dizapam to control FS relapse in children up to July 2014. Two reviewers independently screened literature according to the inclusion and exclusion criteria, extracted data and evaluated the methodological quality of included studies. Then meta-analysis was performed using RevMan 5.2 software, in subjects with low-, moderate- and high-risk of FS recurrence respectively.Results Nine studies involving 1 578 patients were included. The methodological quality evaluation defined generation of random sequences for randomization, allocation concealment, and blinding as high bias risk; selective result reporting, loss of follow up, and alternative sources of bias as low bias risk. Meta-analysis showed no significant difference in FS recurrence rate in 6-month treatment group and intermittent diazepam compared with the control group (RR=0.62, 95% CI: 0.34 to 1.13, P=0.12；RD=-0.07, 95%CI: -0.16 to 0.02). However, FS relapse rate was markedly decreased at 12 months treatment (RR=0.59, 95%CI: 0.38~0.91, P=0.02; RD=-0.12, 95%CI: -0.22~-0.02) and 24 months treatment (RR=0.54, 95%CI: 0.37 to 0.78, P=0.001；RD=-0.17, 95%CI: -0.27 to -0.07), compared with the control group. The results of subgroup analysis by FS recurrence risk factors indicated that reduced FS recurrence rate was found in the moderate-risk group (6 months: RR=0.31, 95%CI: 0.15 to 0.62, P=0.000 9;12 months: RR=0.39, 95%CI: 0.20 to 0.75 , P=0.005;24 months: RR=0.43, 95%CI: 0.24 to 0.77, P=0.005) and high-risk group (6 months: RR=0.24, 95%CI: 0.10 to 0.15; 12 months: RR=0.27, P=0.00 09. 95%CI: 0.13 to 0.58, P=0.000 7; 24 months: RR=0.35, 95%CI: 0.19 to 0.62, P=0.000 4) but not in the low-risk group (6 months: RR=0.69, 95%CI: 0.40 to 1.21, P=0.20;12 months: RR=0.81, 95%CI: 0.47 to 1.42, P=0.46;24 months: RR=0.71, 95%CI: 0.45 to 1.11, P=0.14). No serious complications about diazepam was reported in the included studies.Conclusion Intermittent diazepam for 12 months or 24 months can effectively reduce FS recurrence rate, and shows good efficacy for moderate-risk and high-risk patients. Additional high quality studies are still expected to verify the above conclusion.
Objective The activities of folylpolyglutamate synhase (FPGS) and gamma-glutamyl hydrolase (GGH) dynamically determine the intracellular concentrations of methotrexate(MTX) and its active metabolites, methotrexate polyglutamates (MTXPG), in ALL cells, is the key determinant of its antileukemic effects. To investigate whether the expressions of FPGS, GGH and the ratio of FPGS to GGH gene expression between pediatric patients with ALL and the normal control children are different, and if they are correlated with chemotherapeutic efficacy of MTX in ALL patients. Methods The children newly diagnosed with ALL at Shenzhen Children's Hospital were recruited as ALL group, children with nonmalignancy as the control group. Bone marrow samples from ALL group and peripheral blood samples from the control group were analyzed for the expressions of FPGS and GGH genes by SYBR Green relative quantitative real-time PCR. The difference of FPGS and GGH gene expressions was compared between ALL group and the control group. The subjects were divided into high- and low-expression subgroups according to the median of FPGS and GGH expression level，the discrepancies of MTX effect, toxicity and prognosis were analyzed by different expression subgroups of FPGS and GGH.Results Sixty-one children with newly diagnosed ALL at mean age 4.6 years were enrolled in this study, including 39 males and 22 females; thirty children averagely aged 4.2 years were enrolled in the control group, including 19 males and 11 females. The median (P25-P75) expressions of FPGS, GGH and the ratio of FPGS to GGH gene expression in ALL group（FPGS/β-actin, GGH/β-actin, FPGS/GGH）were 14.14(6.52,27.31)，9.34(4.97,14.22) and 1.74(0.76,2.91), significantly higher than 0.59(0.35-1.23)，3.00(0.94-5.26) and 0.26(0.13-1.00) in the normal group(P＜0.01). The recurrence rate was 3.7% (1/27) in the subjects with higher FPGS gene expression verse 23.3% in those with lower gene expressions (P<0.05). High expression of FPGS gene was correlated with higher risk of neutropenia（OR=4.167，95%CI：1.65-10.52，P=0.003）. High expression of GGH increased the risk of hepatotoxicity（OR=5.61，95%CI =1.14-27.47，P=0.033). No correlation was found between the ratio of FPGS to GGH gene expression and chemotherapeutic effect, toxicity of MTX in this study.Conclusion The expressions of FPGS, GGH and the ratio of FPGS to GGH gene expression in ALL children were higher than that in the control children. Expression of FPGS gene is associated with chemotherapeutic effect, toxicity of MTX in ALL children.
Objective To investigate the association between polymorphisms of Toll like receptor 2 (TLR2) gene and tuberculosis (TB) in Chinese Han children.Methods A case-control study was performed in TB patients obtained from pediatric patients with TB admitted to Beijing Children′s Hospital between February 2005 and October 2010, and other lung infection was excluded. TB patients were diagnosed as pulmonary TB (PTB) , extrapulmonary TB (EPTB), severe tuberculosis (SevTB) and non-severe tuberculosis (NonSevTB). Healthy controls were performed in health examination Chinese Han children, and TB infection was excluded. MassARRAY was used to find the difference of 5 SNPs of TLR2 gene, including rs3804099 (allele C/T), rs1898830 (allele A/G), rs7656411 (allele G/T), rs11935252 (allele A/G), rs7696323 (allele C/T) and genotyping between TB group and the healthy control group. Then the difference of genotypes between TB and the control groups detected with dominant heredity model and recessive heredity model was analyzed.Results ①TB group included 435 children, the mean age was 5.6 years(SD 4.7),male(n=219, 63.5%);PTB(n=151,43.81%),EPTB(n=194);sevTB(n=155,44.9%),NonsevTB(n=190).The control group enrolled 385 children with mean age 6.1(SD 3.8) years, males (n=226, 58.7%). Genotype frequen-cies of 5 SNPs in cases, controls and all subjects met Hardy-Weinberg equilibrium. No statistically significant differences in allelic and genotypic distributions of 5 SNPs were observed between TB and control groups(P=0.154-0.992). After adjustment by age and gender, the genotype of rs3804099 (TC,CC) and the frequency of allele C were found no statistical significance compared with the control group(0.929-1.071); rs1898830 (GA,GG) and the frequency of allele G were also found no significant difference compared with the control groups(OR=1.110-1.411); rs7656411 (GT,TT) and the frequency of allele T were found no statistical significance between TB and control group(OR=0.812-1.110)；rs11935252 (GA,GG) and the frequency of allele A were found no significant difference compared with the control group (OR=0.497-1.189) and rs7696323 (TT,CT) and the frequency of allele C were found no statistical significance compared with the control group (OR=0.888-1.034)(P>0.05) too. ② Neither in the dominant heredity model nor recessive heredity model, the genotype distribution of the 5 SNPs in cases group significantly differed from that in the control group, either in PTB subgroup or in EPTB subgroup (OR=0.737-1.326). And same results were found in sevTB and nonsevTB subgroups (OR=0.675-1.248), P>0.05.③No LD blocks were identified in the 5 SNPs′ of TLR2.Conclusion SNPs of TLR2 genes were not associated with TB suseptibility in Chinese Han children.
Objective To investigate the association between television viewing time and dietary patterns among preschool children.Methods All the children and their parents from 11 kindergartens in Hongkou District, Shanghai China were included. The Socio-demographic questionnaire was used to collect the basic information and television viewing time of the children. Dietary information was collected by using a simplified food frequency questionnaire (FFQ), which included children′s common food intake during the past week. Principal component analysis was used to derive two dietary patterns based on the FFQ. Children′s weight and height were also measured uniformly and BMI was calculated. Children′s television viewing time was divided into 2 groups using 2 hours as the cutoff point and to explore the association between television viewing time and dietary patterns.Results The final sample consisted of 1 670 children，boys accounted for 49.3%. And average BMI was （15.8±1.8） kg·m-2 and BMI Z score was 0.015±0.96. ①On weekdays children who watched TV more than 2 h·d-1 were 12.9%，while at weekends were 36.6%. ②Two dietary patterns were labeled as "the traditional dietary pattern" and "the western dietary pattern". The former included fruits, vegetables, red meat，white meat and staple food，whereas the latter included snack, juice, soft drinks and white meat. ③Comparing with children watching TV less than 2 h·d-1, children spending more than 2 h·d-1 watching TV seemed to have lower traditional dietary pattern scores but higher western dietary pattern scores, even after adjustment for age, gender, BMI levels, and family income. ④Preschool children with higher western dietary pattern scores appeared to have higher BMI（β= 0.066，P=0.006）.Conclusion Western dietary patterns might be the risk factors for preschooler′ obesity. Shorter television viewing time may be beneficial for pre-school children′s dietary patterns. Children who have excessive television viewing time may need to be encouraged to consume a more healthy diet
Objective To enhance the understanding of clinical characteristics，diagnosis，follow-up and genetic testing of chromosome 16p11.2 deletion syndrome.Methods The clinical manifestations，laboratory testing，diagnosis，follow-up，and genetic testing of one case with chromosome 16p11.2 deletion syndrome were reviewed，analyzed and summarized. Meanwhile, relevant literatures of chromosome 16p11.2 deletion syndrome were reviewed in this article.Results ①A 2-month-and-13-day boy with 20-day fever，cough，and diarrhea was admitted to our hospital. Deformity of six fingers in right palm and scoliosis was found. The total peripheral blood lymphocytes and lymphocyte subsets were lower than the reference levels. Chest X-ray indicated that the sternum shape was abnormal and T9-T12 vertebral bodies were hemivertebrae deformity. The patient was improved with a hyperactive and exciting performance after anti-infection therapy. Follow up after releasing indicated that the count of peripheral blood lymphocytes was improved, however, WBC, N and CD4+ T cells remained low levels. The boy was diagnosed as epilepsy at 5 months old and improved after treatment with anti-epileptic drugs. A deletion of 0.545 4 Mb in chromosome 16p11.2 was identified by chromosome chip detection technology and confirmed by high-density oligonucleotide comparative genomic hybridization (CGH) Microarray. The genes located in this deleted region included SPN, QRRT, C16orf54, KIF22, MAZ, SEZ6L2, CDIPT, ASPHD1, KCTD13, TMEM219, TAOK2, DOC2A, TBX6. The results of Chromosome chip detection were normal in his parents. Thus, this boy was finally diagnosed as chromosome 16p11.2 deletion syndrome. ②1 387cases were reported by 95 published articles related with chromosome 16p11.2 deletion syndrome, involving the nervous system（547,39.7%）, endocrine system（371,26.9%）, growth and skeletal abnormalities(84，6.1%), urinary and digestive system(10,0.7%)，cardiovascular system（4,0.3%）, immune function（1,0.07%). The different size of the deletion region in chromosome 16p11.2 led a high heterogeneity of clinical characteristics.Conclusion Chromosome 16p11.2 deletion syndrome has variable clinical manifestations ，including multiple skeletal deformities(such as scoliosis), nervous system abnormalities(such as seizure, autism), other systems(such as repeated infection, endocrine abnormalities). The diagnosis of Chromosome 16p11.2 deletion syndrome relies on chromosome chip detection technology and CGH microarray.
Objective To summarize and review the clinical data of a child with Dent-2 disease so as to improve our understanding to the disease.Methods Clinical data of the case with Dent-2 disease were summarized, including clinical manifestations, laboratory findings, renal pathological changes and family investigation. Mutation analysis in CLCN5 and OCRL genes was performed by direct sequencing in his family. Mutations of CLCN5 and OCRL genes were examined in normal control and related literatures from PubMed were reviewed also.Results Age at onset was 6 years old. Proteinuria was the only symptom at onset. The patient had no extrarenal symptoms of Lowe syndrome, such as peripheral cataracts, mental impairment and stunted growth. Laboratory findings showed low-molecular weight proteinuria, hypercalciuria, microscopic hematuria and elevation of creatine kinase/lactate dehydrogenase. Renal pathological change detection showed a minute lesion under light microscope. A novel homozygous c.260delA (p.Q87fs105X) mutation was found in exon 5 of OCRL gene. No mutations of CLCN5 gene were detected. Mutation analysis showed his mother carried a c.260delA mutation, but his father did not. This deletion caused a frameshift of amino acids and resulted in a truncated protein (p.Q87fs105X), and was not identified in the 100 healthy, unrelated controls.Conclusion Dent-2 disease is an X-linked tubulopathy characterized by low-molecular-weight proteinuria (LMWP), hypercalciuria, nephrocalcinosis, nephrolithiasis, and the development of renal insufficiency. Dent-2 disease can be diagnosed by these characteristics. Lowe syndrome was ruled out in the patient with no peripheral cataracts, mental impairment and stunted growth. Elevation of creatine kinase/lactate dehydrogenase and carrying the homozygous frameshift mutation in OCRL gene support the diagnosis of the patient in the current study. This is the first report of Dent-2 disease caused by OCRL gene mutation in Chinese family.
Background and objective: Network meta analysis has been accepted by health technology assessment agencies in the world, however, how to report it is still a problem. So we conducted a systematic review of available reporting checklist for network meta analysis to guide how to report network meta analysis. Method: The reporting checklist for network meta analysis was identified via searching and compared in order to get the best one. Results: Six reporting checklists for network meta analysis were identified. They were developed by National Institute for Health and Clinical Excellence (NICE)、International Society for Pharmacoeconomics and Outcomes Research (ISPOR）, Dr. Ohlssen from Novartis, Dr. Hutton from Ottawa hospital, Dr. Tan from LeicesterUniversity、Dr. Thorlund from McMaster university. These six checklists did not achieve agreements in reporting network meta analysis. Most of them required to report the method and result sections, but did not require to report the background and discussion section. Conclusion：This study was based on the comparison of six reporting checklists for network meta analysis and explained how to report network meta analysis. However, the reporting checklist for network meta analysis did not achieve agreements and were not good enough. In the future, a better reporting checklist for network meta analysis was required.
Bayesian models were always considered as the golden standard for conducting network meta analysis, however, recent studies showed that both Bayesian and Frequestist methods could generate similar results, but Frequestist methods were easy to be understood without knowing too much statistical knowledge. So this article aimed to review the implementation of Frequestist methods in Stata for network meta analysis.
Childhood primary angiitis of the central nervous system(cPACNS) is a rare disease of central nervous system. The clinical feature is that the pathological changes were limitied in the cerebral with no changes in other parts of body. Diagnosis the treatment of cPACNS are difficult because for the complicated and nonspecific clinical manifestation. This is a case report of a patient with cPACNS. The patient had less physical activities onset with progression gradually. The lesions of arteries were obvious on the magnetic resonance angiography(MRA) of cerebral. And the patient was relieved by prednisone and the pulse of cyclophosphamide(CTX). More researches are needed to improve the diagnosis and treatment of cPACNS.
