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 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.

Child, Preschool ; Epilepsy, Rolandic ; diagnosis ; drug therapy ; physiopathology ; Humans ; Male ; Prognosis ; Steroids ; therapeutic use ; Treatment Outcome

OBJECTIVEWilson's disease (WD) is an autosomal recessive disorder characterized by excessive accumulation of copper in the liver and later in the brain and other organs. Penicillamine acts as a reductive chelator. Zinc salts induce the synthesis of metallothionein in cells. Thus these two drugs are theoretically synergistic for the treatment of the disease. However, the two drugs may also have some unfavorable interactions. In this study, the effect of the therapy with combined penicillamine and zinc salts was evaluated based on the follow-up observations of 21 patients with Wilson's disease.

METHODSUsing the combined therapy of penicillamine [10-30 mg/(kg.d)] and zinc (22.5 mg, 3 times per day), follow-up study by hospitalization or communication with telephone or mail.

RESULTSBefore treatment, all the 21 patients were suffered from chronic liver disorder. Among them, 13 patients (62%) showed to be reactive to the treatment for their liver disorder, 5 patients (24%) died, and 3 patients (14%) dropped off our follow-up study. Among the 5 patients who died, 3 died within 40 days after treatment, one had taken penicillamine only 8 mg/(kg.d), and one died after discontinuation of the treatment by the parents. Of the 12 patients having neurological involvement, neurological symptoms disappeared or markedly improved in 11 patients after treatment. One patient dropped off the follow-up study. The patient with renal tubular acidosis responded well to the treatment. Urine routine analysis was followed up in 6 of the 7 patients with hematuria. Hematuria disappeared in one, became less severe in 1, and remained unchanged in 4 patients. Hypersensitivity to penicillamine was found in one patient. WBC and platelet were found decreased further in 3 patients after the medications.

CONCLUSIONSThe combined therapy with penicillamine and zinc salts was effective in treatment of patients with Wilson's disease.

Adolescent ; Antidotes ; administration & dosage ; adverse effects ; therapeutic use ; Child ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Hepatolenticular Degeneration ; drug therapy ; Humans ; Male ; Penicillamine ; administration & dosage ; adverse effects ; therapeutic use ; Treatment Outcome ; Zinc ; administration & dosage ; adverse effects ; therapeutic use

[ ABSTRACT] AIM:To investigate the effect of oxidized low-density lipoprotein ( ox-LDL) on autophagy in mac-rophages and the underlying molecular mechanisms.METHODS:RAW264.7 macrophages were pretreated with 2 mg/L anti-CD36 monoclonal antibody (anti-CD36 mAb), 5 μmol/L diphenyleneiodonium (DPI), 3 mmol/L 3-methyladenine (3-MA) or 1μmol/L rapamycin for 1 h and then treated with ox-LDL (100 mg/L) for 12 h.The viability of the cells was measured by MTT assay.The activities of lactic dehydrogenase ( LDH) in the medium and nicotinamide adenine dinucleoti-de phosphate ( NADPH) oxidase, superoxide dismutase ( SOD) in the cells as well as the levels of intracellular reactive ox-ygen species ( ROS) and malondialdehyde ( MDA) were determined to characterize the membrane integrity and the oxida-tive stress, respectively.The protein levels of beclin-1 and microtubule-associated protein 1 light chain 3-II ( LC3-II) , 2 important molecular markers of autophagy, were examined by Western blotting.RESULTS:ox-LDL induced autophagy in
RAW264.7 macrophages as assessed by upregulation of beclin-1 and LC3-II.Similar to 3-MA, an autophagy inhibitor, an-ti-CD36 mAb significantly inhibited the ox-LDL-induced upregulation of beclin-1 and LC3-II.Anti-CD36 mAb suppressed the ox-LDL-induced oxidative stress as revealed by decreased NADPH oxidase activation, ROS and MDA generation as well as increased SOD activity.Similar results were observed in the cells pretreated with DPI, a NADPH oxidase inhibitor.Mo-reover, DPI significantly inhibited the ox-LDL-induced upregulation of beclin-1 and LC3-II.Inaddition, the decrease in the cell viability and increase in LDH release induced by ox-LDL were promoted by 3-MA and blocked by rapamycin ( an auto-phagy inducer).CONCLUSION: ox-LDL induces autophagy in RAW264.7 macrophages, which may be involved in CD36-mediated ox-LDL uptake and subsequent activation of oxidative stress, and moderate activation of autophagy may pro-tect macrophages from ox-LDL-induced injury.

OBJECTIVE:To compare the efficacy,safety,vascular endothelial growth factor(VEGF)and matrix metallopro-teinase-2 (MMP-2) of docetaxel combined with carboplatin and paclitaxel combined with cisplatin (DDP) in the treatment of ad-vanced ovarian cancer. METHODS:120 patients with advanced ovarian cancer were randomly divided into docetaxel combined with carboplatin group(60 cases)and paclitaxel combined with DDP group(60 cases). Docetaxel combined with carboplatin group received 70 mg/m2 Docetaxel injection,intravenous infusion of 1 h,d1;50 mg/m2 carboplatin injection,intravenous infusion of 1 h,d2. Paclitaxel combined with DDP group received 135 mg/m2 Paclitaxel injection,intravenous infusion of 24 h,d1;30 mg/m2 DDP for injection,intravenous infusion,d3;60 mg/m2 Paclitaxel injection (a maximum of 2.0 m2) by intraperitoneal infusion,d8. 3-week was regarded as 1 treatment course,and it lasted 6 courses. Clinical efficacy,VEGF,MMP-2,progression-free survival, overall survival before and after treatment,mortality rate within 2 years of treatment and the incidence of adverse reactions in 2 groups were compared. RESULTS:There were no significant differences in the objective response rate,disease control rate,mortal-ity rate,incidence of adverse reactions between 2 groups(P>0.05). The progression-free survival in docetaxel combined with car-boplatin group was significantly longer than paclitaxel combined with DDP group,the difference was statistically significant (P<0.05). Before treatment,there were no significant differences in VEGF and MMP-2 level between 2 groups(P>0.05). After treat-ment,VEGF and MMP-2 level in 2 groups were significantly lower than before,and VEGF at different time points and MMP-2 level after 4 weeks,8 weeks and 12 weeks of treatment in docetaxel combined with carboplatin group were lower than paclitaxel combined with DDP group,the differences were statistically significant(P<0.05). CONCLUSIONS:Docetaxel combined with car-boplatin and paclitaxel combined with DDP shows similar efficacy and safety in the treatment of advanced ovarian cancer,but docetaxel carboplatin combined with is superior to paclitaxel combined with DDP in reducing VEGF and MMP-2 and improving pro-gression-free survival.

Objective To explore the incidence,clinical status,risk factors and outcomes of invasive fungal infections(IFIs)after allogeneic hematopoietic stem cell transplantation (Allo-HSCT) in pediatric patients.Methods Forty-one Patients who were underwent Allo-HSCT were selected from 2005 to 2006. Of 41 patients, 24 were boys and 17 were girls,aged 2-13 years old. Twenty-six cases with ?-thalassemia, 1 case with adrenoleukodystrophy,and the left 14 cases with other hematologic disorders.Twenty patients underwent bone marrow transplantation,19 patients underwent peripheral blood stem cell transplantation,2 patients underwent bone marrow transplantation and cord blood transplantation.Fourteen patients received Allo-HSCT from HLA-matched sibling donors or HLA mis-matched parents, 27 patients received Allo-HSCT from unrelated donors. Based on different types of transplant, patients were conditioned with busulfan, cyclophosphamide and Anti-thymocyte immune globulin. Fludalabine, total body irration, thiotepa or melphalan was used additionly in some cases. Cyclosporine A and mycophnolate mofetil were used as prophylaxis of graft versus host disease (GVHD).Results IFIs was observed in 5 cases(5/41 cases,12.2%),this comprised cases of proven,probable and possible IFIs at rates of 2.4%,4.9%,4.9%.The time of IFIs was 9-120 d after transplantation,the majority of IFIs in 3/5 cases(60%)children occurred within the first month.The difference of IFIs between patients who recived high-dose corticosteroid and those with no or conventional-dose corticosteroid was significant(?2=8.201 P=0.004);Regarding conditioning regimens,the IFIs of patients who with Thiotepa (TT) was significanthy higher than that of compared with those without TT(?2=9.549 P=0.002).The total effective rate was 40%.The effective rates of the patients with confimed diagnosis,cli-nical diagnosis,and with recommended diagnosis respectively were 100%,0 and 50% respectively.Conclusions IFIs is an important complication after Allo-HSCT,and the high-dose corticosteroid therapy and conditioning regimens with TT are the risky factors for IFIs.Aspergillus is the main pathogen bacteria.

OBJECTIVETo explore the theory of "Fei and Dachang being interior-exteriorly related" and the pathogenesis of lung injury by observing changes of inflammatory cytokines and oxygen free radicals in ulcerative colitis (UC) rats.

METHODSTotally 50 healthy male Wistar rats were randomly divided into two groups, the normal control group and the model group, 25 rats in each group. The UC model was established by allergizing colon mucosa combined with TNBS-alcohol (50%) enema. Another 25 rats were recruited as the normal control group. At week 2 and 4 after modeling, the pathomorphological changes of the lung were observed. Furthermore, the contents of tumor necrosis factor alpha (TNF-alpha) and IL-1beta were determined by ELISA. The activities of superoxide dismutase (SOD) and malondialdehyde (MDA) were evaluated with colorimetry.

RESULTSCompared with the normal control group, the pathomorphology of the lung tissue in the model group appeared abnormal at week 2 and 4. Compared with the normal control group, levels of TNF-alpha, IL-1beta, and MDA in the lung tissue significantly increased in the model group (P < 0. 01) and the activities of SOD significantly decreased (P < 0.05, P < 0.01).

CONCLUSIONTNF-alpha, IL-1beta, SOD, and MDA might be common material bases for the large intestine involved in lung disease of UC patients, thus providing a modern scientific basis for the theory of Fei and Dachang being interior-exteriorly related.

Animals ; Colitis, Ulcerative ; diagnosis ; metabolism ; pathology ; Cytokines ; metabolism ; Interleukin-1beta ; metabolism ; Lung ; metabolism ; Male ; Malondialdehyde ; metabolism ; Medicine, Chinese Traditional ; Rats ; Rats, Wistar ; Reactive Oxygen Species ; metabolism ; Superoxide Dismutase ; metabolism ; Tumor Necrosis Factor-alpha ; metabolism

Adenocarcinoma, Mucinous ; drug therapy ; metabolism ; pathology ; surgery ; Adult ; Aged ; Breast Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Carcinoma, Ductal, Breast ; drug therapy ; metabolism ; pathology ; surgery ; Carcinoma, Lobular ; drug therapy ; metabolism ; pathology ; surgery ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Lymphatic Metastasis ; MCF-7 Cells ; metabolism ; Mastectomy, Modified Radical ; Membrane Proteins ; metabolism ; Middle Aged ; Receptor, ErbB-2 ; metabolism ; Sphingosine N-Acyltransferase ; metabolism ; Survival Rate ; Tumor Suppressor Proteins ; metabolism

A(p.G888S)were detected in POLG1 gene.Sequence analysis of parental blood DNA revealed that her father carried L83P and her mother carried G888S.Conclusions The characteristics of clinical manifestation,electrophysiology,pathology and POLG1 gene mutation of the patient were highly consistent with Alpers syndrome.The prominent white matter change and increased immunological factors in CSF were first reported in Alpers syndrome.Alpers syndrome should be considered for those patients whose liver function were severely impaired after exposure to valproic acid.