Adrenoleukodystrophy ; genetics ; therapy ; Humans ; Lovastatin ; pharmacology

Objective To explore the value of dexamethasone(DEX) for neuronal cell injury and death by observing the effect of DEX on excitatory amino acid(EAA) and monoamine neurotransmitter in cerebral tissue of neonatal rat with hypoxia-ischemia.Methods Hypoxic-ischemic neonatal rat models were established,the levels of EAA and monoamine neurotransmitter in cerebral tissue were analyzed by using capillary electrophoresis and fluorospectrophotometry method.The rats were divided into 4 groups: small dose DEX group pre-treated with DEX(0.5 mg/kg) prior to hypoxia-ischemia,large dose DEX group pre-treated with DEX(10 mg/kg) prior to hypoxia-ischemia,HIE group and shamful operation group.Results The levels of EAA and monoamine neurotransmitter contents in HIE group were significantly higher than those in shamful operation group(P0.05).EAA contents of large dose DEX group greatly decreased compared with HIE group (P

Adolescent ; Child ; Child, Preschool ; Electroencephalography ; Female ; Humans ; Infant ; Retrospective Studies ; Rett Syndrome ; pathology ; physiopathology

0.05).The ratio of TH~+ cells in the experimental group(7.38?0.84)% was higher than that in the control group(0.53?0.17)%,and there was significant statistical difference between the two group(P

Objective To assess the efficacy and safety of ketogenic diet(KD) on refractory epilepsy in children.Methods KD treatment was designed to observe the effects for 12 weeks.Totally 22 children with 16 boys and 6 girls were enrolled in the study.The epileptic syndromes included infantile spasms(13 cases),Lennox-Gastaut syndrome(4 cases),Dravet syndrome(2 cases),and the unclassified(3 cases).The KD was prepared according to the modified Johns Hopkins regimen.Urinary ketones were measured every day to ensure that ketosis state and parents′ diaries were kept to find out when it started to work and the change of seizure frequency.Effects of KD was evaluated by Engel standard.Blood chemistry was done at baseline,4 weeks and 12 weeks to analyze the effects of KD on metabolism.Side effects were monitored and treated.Results All cases completed the KD regimen for at least 2 weeks,19 cases for at least 4 weeks,and 10 cases for at least 12 weeks.Sixteen out of 22 children experienced the seizure reduction within 3 weeks(1-15 d),especially in the first week,and seizure free appeared within 5 weeks(1-32 d) in 8 cases.Overall,the diet achieved the seizure-free in 36.4%(8/22 cases) and an over 90% of seizure frequency reduction in 22.7%(5/22 cases).The efficacy of KD seemed not correlated with the sex,age,etiopathogenisis,and syndromes and so on.Blood chemistry suggested a normal range of glucose level at 4 weeks,though higher than that at the baseline.The blood triglyceride and total cholesterol level at 12 weeks increased strikingly,even beyond the normal range compared with the baseline.The side effects mainly including transient gastrointestinal symptoms and metabolic disturbances were mostly tolerable.Conclusions KD is probably a feasible therapy on refractory epilepsy in children,with quick and high efficacy and few side effects.

Objective To analyze the CT findings of endobronchial spread in lung adenocarcinoma. Methods The CT findings of 15 lung adenocarcinomas or bronchioloalveolar carcinomas with endobronchial spread were reviewed,the distribution and the progression of the spread were evaluated.Results All of the primary tumors were consolidation form.The spread lesions distributed in one side of the lung or both sides along the bronchus.The pleural surface was spared.The CT findings of the spread included centrilobular nodules(n=5),tree-in-bud(n=7),acinar nodules(n=2),ground-glass opacities(n=10)and air- space consolidations(n=13)in the first CT examination.5 cases of the spread lesions only presented centrilobular nodules(single form)and 10 cases presented several appearances(complex form).All of the cases were diagnosed as tuberculosis or pneumonia,and antituberculotic or antibiotic therapy was taken with no effect.The follow-up CT scans showed progression in all cases,and the spread lesions with single form became multiple consolidations.The spread lesions with complex form deteriorated faster than the single one. Conclusion Although the CT findings of the endobronchial spread of the lung adenocarcinoma is specific, the clinical history and laboratory examination also are important for the differential diagnosis with tuberculosis and other infectious diseases.

Objective To explore the initial clinical and imaging characteristics of basal ganglia germinoma in children.Methods Four patients with basal ganglia germinoma were reported.Their clinical features,laboratory findings,radiological manifestations,treatment and outcome were analyzed.They recieved radiation therapy and chemotherapy after diagnosis.All patients were clinically diagnosed,according to the results of low-dose cranial irradiation.The outcomes were followed up for 2 years.Results All patients were male and school-aged(9-13 years) children.The course of the disease ranged from 5 to 13 months.All patients were presented with slowly progressive hemiparesis,and 2 cases of them were presented with cognitive decline and psychosis.Seizure occurred in 2 patients.The serum ?-human chorionic gonadotropin(?-hcG) level was significantly increased in 2 patients(30.16 IU/L and 77.85 IU/L,respectively),and mildly elevated in 1 patient(4.29 IU/L),while serum ?-hcG level in another case was within normal control range.MRI demonstrated mildly high intensity in the left or right basal ganglia on T1-weighted and T2-weighted images without remarkable occupying lesion.Ipsilateral hemiatrophy of the hemisphere and midbrain was also noted.Inhomogeneous Gd-DTPA enhancement was observed.All patients had been treated with radiation therapy and chemotherapy.During 2 years follow up,significant improvement was observed in all patients after therapy,imaging lesions disappeared and the elevated ?-hcG level of those elevated before therapy returned to normal.Conclusions Early diagnosis and treatment for basal ganglia germinoma are critically important to improve the prognosis.In young male patients with progressive hamiparesis,basal ganglia germinoma should be considered for differentiation,if abnormal high intensity signals in basal ganglia on T1-weighted and T2-weighted image with ipsilateral hemiatrophy of the hemisphere are demonstrated on MRI,even without occupying effect.

The stability of microbial flocculant (MBF) produced by Aspergillus sojae and its application to municipal thickened sludge dewatering were studied. The results showed that the MBF had high heat and acid-base endurance with high flocculating activity in a wide range of pH from 1.5 to 12. The MBF retained 96% of flocculating activity after 35 days preservation at 4℃, but in different pH the flocculating activity difference was very apparent after 35 days store at room temperature. The experimental results also demon- strated that the MBF was better than PAM and PAC in reducing specific resistance filtration. The optimal dose of MBF used for intensifying thickened sludge dewatering is 7%(volume fraction). And the more the volume of sludge is treated, the less the cost of MBF for unit volume sludge treatment.

Background Even though the change in wavefront aberrations with correction modality is well documented in the literature,little is known about the underlying mechanism.Complete understanding of the causes responsible for the wavefront change in the combined lens-eye system is important since it provides basic knowledge for further improving the technique to correct refractive error by correcting lenses.Objective The aim of this study was to investigate the influence of refractive correction lens on optical property of the eye by analyzing Zernike aberrations in myopic eyes with contact lens correction.Methods This study was approved by the Ethic Committee of Wenzhou Medical College.Written informed consent was obtained from each subject before entering this study.Zernike aberrations of 52 myopic eyes of 26 subjects with the spherical equivalent-1.75 to-8.50 D were measured using a Hartmann-Shock wavefront sensor.The human eye aberrations were examined at the uncorrected condition,rigid-gas-permeable contact lens (RGP-CL) corrected condition and soft contact lens (Soft-CL) corrected condition.The differences of wavefront aberrations and Zernike coefficients were compared by repeated measurement of single factor variance analysis,and correlation of the aberration changes between uncorrected condition and RGP-CL corrected condition or Sofi-CL corrected condition,between the right eyes and left eyes in different conditions were analyzed by Pearson linear correlation.Results Mean total root-mean-square (tRMS) was (0.71 ± 0.30)μm,(0.54±0.19)μm and (0.74±0.32)μm in the uncorrected condition,RGP-CL corrected condition and Soft-CL corrected condition,with a significant difference (F =8.758,P＜0.001),and tRMS was significant declined under the RGP-CL corrected condition compared with uncorrected condition (t =2.746,P =0.008),and tRMS in RGP-CL corrected condition was significantly lower than that in Soft-CL corrected condition (t =3.428,P =0.001).The high RMS (hRMS) was (0.34±0.12) μm,(0.28 ±0.12) μm,(0.40±0.14) μm in the uncorrected condition,RGP-CL corrected condition and Soft-CL corrected condition,with a significant difference among them (F =10.681,P＜0.001).An insignificant decrease of hRMS was seen in the RGP-CL corrected condition compared with uncorrected condition (t =1.987,P=0.053),but hRMS value was significant higher in the Soft-CL corrected condition than that in the uncorrected condition (t=2.101,P=0.041) and RGP-CL corrected condition (t=4.266,P＜0.001).Compared with uncorrected condition,the axis astigmatism (C5) and spherical aberration (C12) in the RGP-CL corrected condition and spherical aberration (C12) in the Soft-CL corrected condition were significantly reduced (P＜0.05),and the absolute values of trefoil (C6),vertical coma (C7) and tetrafoil (C10) in the RGP-CL corrected condition were lower than those of the uncorrected condition,but vertical coma (C7) absolute value in the Soft-CL corrected condition was increased (P＜0.05).A significantly positive correlation was seen in the spherical aberration (C12) between the RGP-CL corrected condition and uncorrected condition (r =0.763,P＜0.001),and less significant correlation was in the secondary astigmatism (C11) between the Soft-CL corrected condition and uncorrected condition(r=0.469,P＜0.001).Conclusions Different contact lens corrected conditions exert their effects on ocular wavefront structure due to its unique interaction with the eye.RGP-CL wearing has strong modification on wavefront aberrations probably due to its molding effect on corneal surface,which reduces the bilateral symmetry.High order wavefront aberration can be modified by Soft-CL wearing.

Objective To investigate the clinical and electroencephalogram (EEG) characteristics of children with electrical status cpilepticus during sleep (ESES), and the response to medication therapy. Methods AEEG and VEEG including an entire sleeping c-ycle were performed on 26 patients with ESES. The clinical and EEG changes, neuropsychological impairment and the response to medication therapy were followed up. Results Twenty five patients had seizures,21 cases had normal psychomotor development before ESES. After the onset of the disease,Fifteen cases developed language disorder, 16 cases developed psychological and behavior abnormalities, 13 cases had both of the problems Seventeen patients belonged to epileptic syndrome. Patients in this cohort had good response to clonazepam and valproate treatment. Cortical steroid could dispel the electrical discharge. Eighteen patients had been followed up. Seizures stopped in 15 cases after treatment ESES disappeared in 16 cases, 4 of them still had neuropsychological impairment ESES sustained in 2 cases Conclusions ESES is a specific EEG phenomenon. Continue epileptic form discharge during non - rapid cye movement sleep is the major cause of neuropsychological impairment in patients with ESES. To control the seizures and electrical state are very important for the prevention and treatment of neuropsychological impairment.