Objective To investigate the association of hepatitis B virus(HBV) S gene quasispecies with the outcome of HBV infection.Methods Serum samples were collected from three chronic HBV carriers, three chronic hepatitis B and three chronic severe hepatitis B patients.All subjects were male and with HBV genotype C.HBV S gene was amplified, and 20 clones of HBV fragment were randomly selected and sequenced from each sample.SPSS 15.0 software was adopted for analysis.Results Quasispecies complexity of HBV S gene in chronic HBV carriers and chronic hepatitis B tended lower than that of the severe chronic hepatitis B, but the difference was not of statistical significance (P＞0.05).In T cell epitope 45, 47, 85 amino acid sites of the HBV S gene, the constitution of quasispecies in the chronic hepatitis B was more complex than that of the HBV carriers (P=0.01), but compared with the severe chronic hepatitis, the difference was not significant (P=0.06).The computer model showed that both the dominant clones and the non dominant clones could effectively bind to the receptors of cytotoxic T lymphocytes.Conclusion Quasispecies in some T cell epitopes of HBV S gene may be related with the clinical outcome of hepatitis B.

We originally created the theory of "four separated" management of goods, by which we achieved the effective administration in the aspects of check and approval, purchase, safekeeping and usage of materials in our hospital. Combining with practical work of our hospital, this article analyzed and summarized the theory, application, effect and significance of the "four separated" management of the goods.

BACKGROUND: Growth factors and other biological methods have become very popular in the repair of degenerative interevrtebral disc. Insulin-like growth factor-1 (IGF-1) can promote the proliferation of nucleus pulposus cells, and synthesis of functional extracellular matrix, but the mechanisms remain unclear.OBJECTIVE: To investigate the effect of IGF-Ⅰ on the expressions of aggrecan and collagen type Ⅱ in nucleus pulposus cells, and to explore its signal transduction mechanism.METHODS: The human nucleus pulposus cells were isolated and cultured. Passage 3 nucleus pulposus cells were induced in different concentrations of IGF-1 (0, 20, 50, 100 and 200 μg/L), respectively. The expressions of aggrecan and collagen type Ⅱ were detected by reverse transcription PCR and western blot assay. Western blot assay was adopted to observe the effect of 100 μg/L IGF-1 on the activation of PI3K/Akt signaling pathway in nucleus pulposus cells,and the expression of aggrecan and collagen type Ⅱ was detected after the inhibition of PI3K/Akt pathway by LY294002.RESULTS AND CONCLUSION: With the increase of IGF concentration, the expression levels of aggrecan and collagen type Ⅱ were increased gradually. 100 μg/L IGF-1 could significantly promote the expressions of p-PI3K and p-Akt (P <0.01), while LY294002 reversed this effcet (P < 0.01). 100 μg/L IGF-1 significantly upregulated the expression levels of aggrecan and collagen type Ⅱ in nucleus pulposus cells (P < 0.01); in contrast, LY294002 significantly downregulated the expression levels of aggrecan and collagen type Ⅱ promoted by IGF-1(P < 0.01). These results indicate that IGF-1 can promote the expression levels of aggrecan and collagen type Ⅱ in nucleus pulposus cells via the PI3K/Akt signaling pathway.

Objective To explore the efficacy of ketogenic diet( KD) in the treatment of status epi-lepticus( SE) and whether KD could protect the brain,and propose a new thought on SE patients′reasonably individualized treatment, brain protection and prognosis improvement. Methods From Sep 2013 to Jan 2015,all the patients diagnosed as SE were advised to apply KD treatment; the patients who refused KD treatment were included in the control group,while the patients who accepted KD treatment were included in the treatment group. Based on the SE treatment principles,the control group applied traditional anti-convulsive therapy,while the treatment group applied traditional therapy combined with KD treatment. Before the treat-ment and after the epilepsy control,the patients′ serum was collected to test neuron specific enolase( NSE) and S100βlevels,and the duration of epilepsy control was recorded. Results The treatment group included a total of 10 patients; 3 patients had a good efficacy and obtained seizure-free after the treatment; clinical seizures declined significantly in 6 patients. The treatment group′s overall response rate was 9/10,which was higher than that of the control group(5/8)(P<0. 01). The treatment group′s duration to gain efficacy was shorter than that of the control group[(5. 2 ± 2. 9) d vs. (9. 8 ± 1. 5) d,P<0. 01]. After the treatment,the patients′NSE and S100β in both groups were significantly decreased than those before the treatment ( P<0. 001 or P<0. 05). After the treatment,the serum NSE and S100β of the patients in the treatment group were lower than those in the control group,with statistically significant difference(P<0. 05). Conclusion Frequent epileptic seizures and SE would impair the patient′s brain. Controlling the epileptic seizures actively could lower the severity of brain injury. KD could effectively control the epileptic seizure and had neuropro-tective effects.

Objective To analyze the clinical manifestations of familial acute necrotizing encephalopathy (ANE)and to improve the recognition of this disease. Methods The clinical data of a 25 - month - old girl with fa-milial and recurrent ANE with evidence of mutation in the RANBP2 gene were collected and analyzed,and the gene examination of their family members was performed. Results A previously healthy girl experienced recurrent ANE epi-sodes at the ages of 8 months,18 months and 25 months,respectively. At each beginning of each episodes the patient presented with lethargy and tremor of limbs following febrile illness of 3 - 4 days,even developed coma and convulsions in the last time. Brain magnetic resonance imaging showed bilateral and high T2 signal changes in thalamus,cerebellum and hippocampus. Abnormal signals also appeared in the brainstem,claustrum,corpus scallosum and cortex(temporal, parietal and cingulate)also appeared abnormal signals. Spinal MRI showed spinal cord involvement. The girl recovered after her first episode;she could speak but could not walk steadily after the second time;after the third episode,al-though she regained consciousness from coma,she could no longer speak or walk. The patient's sister died of encephali-tis at the age of 18 months. Her paternal uncle had suffered from dysnoesia from meningitis at his 17 months of age. The patient and her grandmother,father,uncle and one of her aunts harbored a mutation(c. 1754C ﹥ T)in RANBP2 gene. Conclusions Familial ANE has typical clinical manifestations and characteristic MRI findings. The patient with recur-rent history,especially with positive family history,should have the mutation in RANBP2 gene detected earlier in order to clarify the diagnosis of ANE.

Objective To compare the clinical and electroencephalogram(EEG)characteristics and therapeutic response prognosis of different age groups of children with epileptic spasm .Methods From January 2002 to October 2011 the clinical data ,EEG fea-tures of epileptic spasms children under 15 years old with unknown disease cause(cryptogenic) were retrospectively reviewed .74 of them were followed up for 12 to 92 months .All of them were divided into two groups on the basis of onset age :3 -12 months of onset as group infantile-onset spasms(group IOS ,n=60);and 12 months to 7 years old of onset as group late-onset epileptic spasms (group LOS ,n=14) .Clinical process ,seizure semiology and EEG features were compared between two groups .Results Semiologic features of two groups were similar ,but they showed differences in interictal EEG features including the background ,the location of discharges ;The response to drugs between the two groups are also different .Conclusion There are differences between group IOS and group LOS when comparing EEG features and response to drugs .

Objective: To improve the diagnostic accuracy of transthoracic echocardiography (TTE) by analyzing its limitations in diagnosing partial anomalous pulmonary venous drainage (PAPVD). Method: This was a retrospective analysis of PAPVD patients seen at the Children′s Hospital of Fudan University from October 1 2006 to October 1 2016. The echocardiographic data were compared to findings on multi-slice spiral CT (MSCT), cardiac catheterization or surgery. The echocardiography machines used were Philip IE33, GE Vivid 7 and Vivid i with frequency ranging from 5.0 MHz to 7.5 MHz. The cardiac structure was analyzed according to Van Praagh segments. Result: A total of 43 cases of PAPVD were enrolled, male∶ female ratio 20∶23 with average age (27.9±21.4) months. Among them, 3 cases were simple PAPVD and 40 cases had other associated congenital heart diseases. TTE was successful in diagnosing 29 cases (67%) while 14 cases were missed. The diagnostic rate for right pulmonary vein drainage into superior vena cava, right atrium, inferior vena cava were 5/10, 17/20, and 3/5 respectively while left pulmonary vein drainage into left innominate vein was only 1/4. Added TTE images to re-exam the 9 of the 14 missed cases, 5 cases of abnormal drainage from right superior pulmonary vein were diagnosed, while 4 cases of drainage from right lower or left pulmonary vein were only picked up by indirect signs. Conclusion The distance of the pulmonary veins from the routine ultrasound view and the possibility of branch number variation may limit the accuracy of TTE in diagnosing PAPVD, especially for drainage from right lower and left pulmonary vein. But TTE is still the preferred diagnostic method. The diagnostic rate could be increased by paying special attention to non-routine views including the suprasternal fossa, the right parasternal and subcostal area.