Objective To identify the incidence and risk factors that are associated with agitation after general anesthesia from 500 pediatric patients .Methods Data about any kinds of the index from 500 pediatric patients ,ASA Ⅰand Ⅱ ,receiving gen‐eral anesthesia were recorded .such as the age ,sex ,weight ,anxious before the anesthesia ,kinds of the disease ,class of ASA ,kinds and dosage of the anesthesia drug ,such as ketamine ,propfol ,sevoflurane ,fentanyl ,the intubation ,length of anesthesia time ,the re‐covery time and the agitation grade .According to the agitation grade ,all the patients divided into two group ,the agitation group and the non‐agitation group .Logistic regression with a descending stepwise algorithm was performed to identify independent variables . The discriminative power of the Logistic regression model was checked with a receiver operating characteristic curve .Results Agi‐tation meeting the predefined criteria occurred in 153 patients(30 .6% ) .The Logistic analysis identified the following varables to be associated with agitation after the anesthesia:age ,sex ,weight ,anxious before the anesthesia ,the use of the ketamine and the sevoflurane ,the intubation .The agitation correlation coefficients for the age ,sex ,and the use of the ketamine were negative ,other‐wise the weight ,anxiety ,the use of sevoflurane and the intubation were positive .The area of ROC was 0 .850 .Conclusion This study contributes to the identification of patients with a high risk for agitation after general anethesia .The index of the age ,sex , weight ,anxious before the anesthesia ,the use of the ketamine and the sevoflurane ,the intubation should be comprehensive evalua‐ted .

Objective To study features of the MRI and clinic in a family with pure hereditary spastic paraplegia (PHSPG) type 6.Methods Target loci (SPG3, 4, 6, 8 10 and 12) linkage analysis was performed in a SPG pedigree having 6 affected individuals using microsatellite markers and NIPA1 gene was screened for mutation by PCR-amplification and sequencing. MRI of brain and cervical and thoracic spinal cord were examined in these 6 patients and 6 normal controls matched for age and sex by two independent radiologists blinded to the clinical diagnosis. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at the levels of C2～3, C7, T1～4, T9 were measured and data was statistically analyzed using the student's t test. Results A missense mutation of 316g→c in NIPA1 was identified in the affected subjects, presumably resulting in substitution of glutamic acid for arginine in residue 106. Evaluation of the brain MRI images revealed non-specific brain abnormalities. All patients presented thinning of cervical and upper thoracic spine with atrophy in both gray and white matter and enlarged subarachnoid cavity. In severe atrophic segments, a distinct boundary between grey and white matter was observed and the lesions in grey matter presented literal high intensity spots or patches with clear boundary on transaxial T2-weighted images (T2WI) and high signal intensity longitudinal strip on the sagittal T2WI. Cross-sectional areas and anteroposterior and transverse diameters of the spinal cord at C2～3, C7, T1～4 were significantly smaller in patients than in controls, while at the T9 level only transverse diameter showed significant difference (7.22±0.08 vs 8.17±0.41, t=2.870, P=0.046). Conclusions These findings indicate that the disease process in patients with SPG6 might be confined to the cervical and thoracic spinal cord, with atrophy in both white and grey matter having a distinct boundary.

Objective To investigate the characteristics of clinical manifestations and genetics of late-onset cobalamin (cbl) C deficiency,also named as combined methylmalonic acidemia and homocystinemia, cblC type. Methods We reviewed 26 late-onset cblC deficiency patients diagnosed in Qilu Hospital, Shandong University from 2012 to 2017 and analysed the clinical, biochemistry, neuroimaging, follow-up and MMACHC gene data. Results Among the 26 patients, male:female ratio is 11:15, with the age of diagnosis from 4 to 39 years and sibling comorbidity in 4 families. The clinical manifestaions of nervous system included spastic paraplegia,mental and behavior disorder,intelectual decline,epilepsy,ataxia,dystonia and peripheral neuropathy. There were four cases with proteinuria at onset. At first visit, the levels of serum total homocystinuria of all patients were elevated, from 61.4 to 193.4μmol/Lwith methylmalonic acidemia. The neuroimaging data of the 26 cases showed 11 with cerebral atrophy, 10 with thoracic spinal cord atrophy, five with brain parenchymal lesions, three with longitudinal myelopathy which were reversible in follow-up, one with syringomyelia, one with multiple cerebral artery stenosis. In all the cases, cobalamins were supplied parenterally and folate, betaine, L-carnitine, vitamin B6 were supplied orally during acute metabolic crisis, and the symptoms of acute encephalopathy disappeared but symptoms of spastic paraplegia had little improvement. In chronic stage, frequency of intramuscular injection of hydroxocobalamine could be decreased while the index can still be improved. All the 26 cases had definite mutations in MMACHC gene, the most common mutations of which were found to be c.482G>A(15/52) and c. 609G>A(13/52). Conclusions Homocystine is the important biomarker for cblC deficiency. Once diagnosed, parenteral hydroxocobalamin and oral betaine should be supplied for a lifetime with good prognosis. The most common mutations of MMACHC gene in our cases are c. 482G>A and c. 609G>A missense mutations.

Objective: To investigate the clinical characteristics and the effectiveness of surgical treatment of adrenal cysts. Methods: The clinical data of adrenal cysts in Affiliated Zhangye people′s Hospital, Hexi College from May 2005 to July 2018 were retrospectively analyzed, including clinical manifestations, diagnosis, surgical methods, postoperative pathological results and follow-up process. Seventeen patients with adrenal cysts were identified including 10 males and 7 females. The median age was 45.6 ranging from 30 to 75. There were 12 left lesions and 5 right lesions. Only 4 patients (23.5%) had clinical symptoms, including low back pain, abdominal pain and dizziness caused by hypertension. Ultrasound, CT and MRI were the main imaging methods for preoperative diagnosis. Results: All patients underwent surgical treatment. Laparoscopic surgery was adapted in 14 cases. The average operation time was (70.2 ± 18.5) min and the average bleeding was (47.0 ± 12.1) ml. For open surgery in 3 cases, the average operation time was (103.5 ± 25.0) min and the average bleeding was (85.6 ± 20.5) ml. Postoperative pathological results showed endothelial cysts in 11 cases, epithelial cysts in 3 cases and pseudocysts in 3 cases. No recurrence of cysts or hypoadrenalism occurred in 3-75 months′ follow-up. Conclusions Adrenal cysts are relatively rare and generally benign lesions. Imaging examination, especially abdominal enhanced CT is essential for diagnosis. Laparoscopic surgery is a safe and effective method for the treatment of adrenal cysts.