Adolescent ; Adult ; Aged ; Endoscopy ; Female ; Humans ; Male ; Maxillary Sinus ; surgery ; Middle Aged ; Otorhinolaryngologic Surgical Procedures ; methods ; Young Adult

OBJECTIVETo summarize the clinical characteristics of alternating hemiplegia of childhood (AHC).

METHODSThe clinical data of 13 children with AHC were analyzed. Cranial MRI, EEG, analysis of serum amino acids and urinary organic acids, measurement of plasma lactate and pyruvate levels were done in all patients. Other laboratory examinations such as magnetic resonance angio-imaging (MRA), digital subtraction arteriography (DSA) and Video-EEG were also performed in some patients.

RESULTSOf the 13 patients, 12 were male, and 1 was female. The age of onset was from 2 days to 55 months (average 13.1 months). The initial symptoms were abnormal ocular movements (AOMs) consisting of ocular deviation, gaze or nystagmus in 2 cases, AOMs and dystonic posturing in 9 cases, hemiplegia in 2 cases. All patients had recurrent alternating hemiplegic episodes. The hemiplegic attacks lasted from a few minutes to 10 days. The occurrence of the attacks ranged from 8 times daily to one time every 2 months. In 10 patients the abnormal eye movements or dystonic posturing, at times, recurred intermittently during the hemiplegic attack. Choreoathetosis was present in 2 cases. Ataxia was present in 1 case; 7 patients also had the episodes of quadriplegia. During the episodes of quadriplegia, dysarthria or aphasia was present in 4 cases, dyspnea and dysphagia was present in 2 cases, respectively. Sleep could relieve both weakness and associated paroxysmal symptoms. Mental retardation was present in 9 cases, seizures in 3 cases. Except for EEG or Video-EEG was abnormal in 3 patients, other laboratory investigations were normal in all patients. Twelve patients received flunarizine therapy. Flunarizine reduced the severity, duration, or frequency of hemiplegic attacks in 8 patients.

CONCLUSIONThe results suggest that AHC is characterized by frequent episodes of alternating hemiplegia with extrapyramidal symptoms and mental retardation, flunarizine is effective in treating some AHC patients.

Anticonvulsants ; therapeutic use ; Child, Preschool ; Electroencephalography ; Female ; Flunarizine ; therapeutic use ; Hemiplegia ; drug therapy ; pathology ; Humans ; Infant ; Infant, Newborn ; Intelligence Tests ; Magnetic Resonance Imaging ; Male ; Treatment Outcome

Ferulic acid (FA) was loaded into liposomes via calcium acetate gradient with (80.2 +/- 5.2)% entrapment efficiency. The average sizes of blank liposome and FA liposome were about 155 nm and 154 nm, respectively. The zeta potential of blank liposome and FA liposome were (13.14 +/- 1.67) mV and (4.12 +/- 0.05) mV, respectively. Unilamellar vesicles were present in freeze-fracture electron microscopy. In the pharmacodynamic studies, the protective effect of liposomal ferulic acid on tBHP-challenged U937 cells was measured with the morphology of cell injury, mitochondrial transmembrane potential alternation and cell viability assay used as index. The results of MTT assay, microscopy indicated that FA liposomes exhibited greater antioxidant activity than FA solution on U937 cell.
Antioxidants ; administration & dosage ; pharmacology ; Cholesterol ; chemistry ; Coumaric Acids ; administration & dosage ; pharmacology ; Drug Carriers ; Humans ; Liposomes ; chemistry ; Membrane Potentials ; drug effects ; Mitochondria ; physiology ; Particle Size ; U937 Cells

OBJECTIVETo provide endoscopic anatomic bony structures of pterygopalatine fossa for skull base surgery.

METHODSThe bony structures of the pterygopalatine fossa were observed in ten dry skulls under endoscope.

RESULTSThe pterygopalatine fossa showed a long and narrow cleft composed of the body and pterygoid process of sphenoid bone, the lamina perpendicular of palatine bone, and the posterior wall of maxillary sinus. The pterygopalatine fossa is (21.4 +/- 0.8) mm x (5.2 +/- 0.3) mm x (3.2 +/- 0.3) mm, with seven paths communicating with nasal cavity, mouth cavity, pharynx, orbit, infratemporal fossa and middle cranial fossa. Under endoscope,the whole pterygopalatine fossa could be observed.

CONCLUSIONSEndoscopic anatomic study of the pterygopalatine fossa is important to endoscopic endonasal skull base surgery. Under endoscope,the whole pterygopalatine fossa can be observed.

Adult ; Anatomy, Regional ; Asian Continental Ancestry Group ; Cranial Fossa, Middle ; anatomy & histology ; surgery ; Endoscopy ; Humans ; Otorhinolaryngologic Surgical Procedures ; Pterygopalatine Fossa ; anatomy & histology ; surgery ; Skull Base ; anatomy & histology ; surgery

In order to detect coagulation factor VIII (FVIII) inhibitor in patients with severe hemophilia A (HA) and preliminarily study the genetic mutation in patients with inhibitor positive. Totally 58 patients with HA (FVIII: C < 1%) were enrolled. FVIII: C activity was measured by one-stage coagulation assay. FVIII inhibitor was screened by using APTT method and FVIII inhibitor in screened positive patients with HA was quantitatively analyzed by using Bethesda method. Using genomic DNA as template, 12, 14, 16 exons of FVIII in screened positive patients were amplified, and the mutations of amplified products were detected by direct sequencing. The results indicated that the FVIII inhibitor could be detected in 4 patients (6.9%) from 58 HA patients, no gene mutations in 12, 14, 16 exons of FVIII were found. It is concluded that the positive rate of FVIII inhibitor in HA patients is lower than that reported in literature. The causes of inhibitor production needs to further investigate.
Adolescent ; Adult ; Blood Coagulation Factor Inhibitors ; isolation & purification ; Blood Coagulation Tests ; Child ; Child, Preschool ; Exons ; Factor VIII ; antagonists & inhibitors ; genetics ; Genetic Testing ; Hemophilia A ; diagnosis ; genetics ; Humans ; Infant ; Middle Aged ; Mutation ; Young Adult

OBJECTIVETo investigate the prognostic factors in non-small cell lung cancer (NSCLC) at stage III and IV and establish a reliable model of clinical prognostic index.

METHODSKaplan-Meier and Cox regression were used to analyze the relationship between the prognostic factors and survival time in 114 cases of NSCLC. The prognostic factors included clinical-pathological features and serum levels of cytokeratin fragment 19 (Cyfra21-1), CEA, neuron-specific enolase (NSE), CA125, interleukin-2 (IL-2) and soluble interleukin-2 receptors (sIL-2R).

RESULTSKaplan-Meier analysis showed that KPS, sex, disease stage, treatment, Cyfra21-1, sIL-2R and CA125 were related to prognosis. Multivariate analysis indicated that Cyfra21-1, stage and treatment were independent prognostic factors. When Cyfra21-1 > 3.5 mg/L, stage IV and chemotherapy < 3 cycles, the relative risk (RR) was 1.691, 2.229 and 3.035, respectively. In patients given 3 or more cycles of chemotherapy, serum Cyfra21-1, sIL-2R and stage at diagnosis were significantly independent prognostic factors. Three of these prognostic factors were used to establish a prognostic index (PI) model based on a simple algorithm: PI = Cyfra21-1 + sIL-2R + stage. The median survival period of patients with 3 or more cycles of chemotherapy were 18 months if PI = 0, 8 months if PI = 1 or 2, and 5 months if PI = 3.

CONCLUSIONThe serum Cyfra21-1, sIL-2R and disease stage in unresectable NSCLC were independent prognostic factors. PI calculated on the basis of Cyfra21-1, sIL-2R and stage is recommended to predict the survival period of NSCLC.

Antigens, Neoplasm ; blood ; Biomarkers, Tumor ; blood ; Carcinoma, Non-Small-Cell Lung ; drug therapy ; mortality ; pathology ; Female ; Follow-Up Studies ; Humans ; Keratin-19 ; Keratins ; Lung Neoplasms ; drug therapy ; mortality ; pathology ; Male ; Middle Aged ; Neoplasm Staging ; Prognosis ; Proportional Hazards Models ; Receptors, Interleukin-2 ; blood ; Survival Rate

Objective To study the needing of different payments of inpatients for nursing operation inform and to provide theoretical guidance for clinical nursing operation inform,so as to meet patients' personalities and diversification needs.Methods Totals of 774 inpatients in four hospitals of Guilin city were investigated with self-designed questionnaire from January to March in 2011,then the results were analyzed.Results Inpatients' needs for nursing operation inform were high both in four groups public expense,selfexpense,basic medical insurance of city,new type of rural cooperative medical care insurance,and especially 85％ inpatients needed most was 11 items of needs.Most of patients selected nurses to be the informer and they were willing to informed in oral way.There were significant difference on the needing of 12 items nursing operations among inpatients with different payments ( P ＜ 0.05 or P ＜ 0.01 ).Conclusions It is necessary to sufficiently assess the needing of different payments inpatients for inform in order to meet their infromed needs of nursing operations.Nurses as important informers,we should pay attention to their role.

OBJECTIVETo investigate the prognostic factors of small cell lung cancer (SCLC) and establish a reliable model of clinical prognostic index.

METHODSKaplan-Meier and Cox regression were used to analyze the relationship between survival time and prognostic factors in 60 cases of SCLC. The prognostic factors included clinical and laboratory parameters, serum cytokeratin fragment 19 (CYFRA21-1), carcinoembryonic antigen (CEA), neuron-specific enolase (NSE), CA125, interleukin-2 (IL-2) and soluble interleukin-2 receptors (sIL-2R).

RESULTSKaplan-Meier analysis showed that poor prognosis was in patients with KPS < 80 or extensive disease and unrelated to other clinical parameters such as age, sex and smoking index, and in patients with serum NSE > 30 micro g/L, CEA > 5.0 micro g/L, CA125 > 37 KU/L and sIL-2R > 500 KU/L. Serum IL-2 and CYFRA21-1 were also elevated, but had no significant prognostic value. Multivariate analysis indicated that serum NSE, stage and treatment of disease were independent prognostic factors. The three prognostic factors enabled establishment of a prognostic index (PI) based on a simple algorithm: PI = NSE (0 if < or = 30 micro g/L, 1 if > 30 microg/L) + stage (0 = LD, 1 = ED) + CEA (0 if < or = 5.0 microg/L, 1 if > 5.0 microg/L).

CONCLUSIONThe stage of disease, systemic treatment and the level of serum NSE are independent prognostic factors. Without considering the influence of treatment-related factors on survival, the levels of serum CEA, NSE and stage of disease before treatment are significant independent prognostic factors. PI calculated on the basis of CEA, NSE and stage is recommended to predict the survival of SCLC.

Adult ; Aged ; Biomarkers, Tumor ; blood ; Brain Neoplasms ; secondary ; Carcinoma, Small Cell ; mortality ; secondary ; therapy ; Female ; Follow-Up Studies ; Humans ; Liver Neoplasms ; secondary ; Lung Neoplasms ; mortality ; pathology ; therapy ; Male ; Middle Aged ; Multivariate Analysis ; Neoplasm Staging ; Prognosis ; Proportional Hazards Models ; Survival Rate

OBJECTIVESevere myoclonic epilepsy of infancy (SMEI), or Dravet syndrome, is a severe epileptic encephalopathy. This study aimed to investigate the clinical features and genetic diagnosis of SMEI.

METHODSThe electroclinical data and the mutation of SCN1A gene in 13 children with SMEI were analyzed.

RESULTSOf the 13 children, 10 were males and 3 were females. Eight of them had family history of febrile seizures. The average age of seizure onset was 5.6 months, with a range of 2 to 9 months. The initial seizure was a febrile seizure in 9 patients (69%). Generalized or hemiclonic seizures were often triggered by fever. Eight patients had a history of febrile status. Afebrile seizures occurred from 2 months to 21 months of age. All patients went on to develop multiple seizure types. Generalized tonic clonus seizures (GTCS) were found in 11, partial seizures in 12, atypical absence in 10. Myoclonic seizures were presented in all patients. Twelve patients had 3 or more seizure types. Seizures of all patients had a characteristic of temperature sensitivity. The precipitating factors included fever, hot bath and vaccination. Nine patients (69%) had a history of status epilepticus. Delay in mental development was present in 11 cases, ataxia in 5 and pyramidal sign in 2. EEG was normal in most patients in the first year of life, followed by generalized, focal and multifocal discharges. Brain MRI was abnormal in 2 cases. Seizures were not completely controlled in all patients. Carbamazepine and lamotrigine aggravated seizures in some patients. SCN1A gene mutation was found in 10 cases, including seven missense mutations, two nonsense mutations and one frame shift mutation.

CONCLUSIONThe clinical features of SMEI were seizure onset within one year of age, first event is often a febrile seizure; multiple seizure types and mental delay occurred after the second year of life; seizures have a characteristic of temperature sensitivity; EEG was normal in the first year of life, followed by generalized, focal or multifocal discharges; early diagnosis by testing SCN1A mutation guides selection of antiepileptic drugs.

Child, Preschool ; DNA Mutational Analysis ; Electroencephalography ; Epilepsies, Myoclonic ; diagnosis ; genetics ; Female ; Genetic Testing ; Humans ; Infant ; Male ; Mutation ; NAV1.1 Voltage-Gated Sodium Channel ; Nerve Tissue Proteins ; genetics ; Sodium Channels ; genetics

OBJECTIVETo establish a bcr-abl(+) cell line resistance to nilotinib, and to investigate the possible mechanisms of resistance.

METHODSK562 cells were treated with gradually increasing concentrations of nilotinib to generate resistance cell line K562-RN. The folder of drug-resistance was evaluated by MTT assay. Cells apoptosis rate was detected by flow cytometry, the mRNA level of bcr-abl fusion gene by FISH, and the expression of apoptosis relative gene mRNA and protein (such as bcr-abl, HO-1, mdr1, Bcl-2 and caspase-3) by RQ-PCR and western blot.

RESULTSThe resistant cell line K562-RN was successfully established, with 2.01 fold resistant to nilotinib compared with K562 cell line \[the IC(50) value of nilotinib to K562 and K562-RN were (12.320 ± 1.720) µmol/L and (24.742 ± 2.310) µmol/L, respectively\]. It also had the cross resistance to adriamycin, homoharringtonine, etoposide and imatinib. Treated with different concentrations of nilotinib, cell apoptosis rate of K562-RN was significantly lower than that of K562 cells. The rate of bcr-abl gene positive cells was 92% in K562-RN by FISH assay. The mRNA and protein levels of bcr-abl, HO-1 and mdr1 expression up-regulated in K562-RN cells, while those of caspase-3 expression down-regulated, being significantly statistical difference when compared with K562 cells (P < 0.05).

CONCLUSIONHuman leukemic cell line resistance to nilotinib, K562-RN is established successfully by gradually increasing concentrations of drug. The mechanisms of resistance in K562-RN is probably associated with increasing expression of bcr-abl, HO-1, mdr1 and decreasing expression of caspase-3 mRNA and protein levels.

ATP Binding Cassette Transporter, Sub-Family B ; ATP-Binding Cassette, Sub-Family B, Member 1 ; metabolism ; Caspase 3 ; metabolism ; Drug Resistance, Neoplasm ; Fusion Proteins, bcr-abl ; metabolism ; Gene Expression Regulation, Leukemic ; Heme Oxygenase-1 ; metabolism ; Humans ; K562 Cells ; drug effects ; Pyrimidines ; pharmacology