Objective To explore the clinical , EMG and genetic characteristics of children with hereditary neuropathy with liability to pressure palsies phenotype (HNPP).Methods One case of HNPP diagnosed by gene were reported, and combined with the literature , the clinical, electromyography and genetic characteristics were summarized.Results Female patient, 11 years and 8 months, left foot could not be dorsal flexion and numbness appeared after the movement in 10 d before admission ,EMG showed multiple peripheral nerve injury .Patient's father and uncle were very similar to the history .Genetic examination showed 1363.2 kb heterozygous deletion on the chr17:14095421 -15458636, and the diagnosis was HNPP .Conclusions When limb weakness happened in children after slight stretch or compression , perform EMG examination as early as possible .Children with extensive peripheral nerve damage , limited limb paralysis , and a similar family history , should pay attention to HNPP , and gene examination should be given .

ObjectiveTo synthesize metal complex that exhibits superoxide dismutase (SOD)-like activity and determine its structure.MethodsThis chemical compound was synthesized by means of reflux in laboratory.Its structure was examined by infrared spectra (IR),nuclear magnetic resonance hydrogen spectra(H-NMR) and mass spectra (MS).The activity of the SOD was determine by xanthine oxidase (XOD).ResultsThe IR indicted that this complex showed a typical absorption band of -OH,C=N and benzene. The H-NMR reveals the existence of metal ion manganese (Mn3+ ,paramagnetic). The molecular weight was 365, which was obtained by EI-MS analysis. And this complex showed SOD-like activity.ConclusionA metal complex with low molecular weight and SOD-like activity has been synthesized.

Objective To study the protective effects of the compound with low molecular weight of anti free radical on brain ischemic and hypoxic injuries in rats.Methods The model of brain ischemic and hypoxic injuries in rats was established by unilateral carotid artery ligature just for 2 h. The compound was injected 30 minutes after the rats act on hypoxic state (10% O2+90%N2) for 1 h. Then the serum of rats was separated and the value of the superoxide dismutase (SOD) activity and malondiade (MDA) were determined, and the pathological changes were observed in brain tissue.Results Compared with rats in the model group, the compound decreased the concentrations of MDA in serum (P<0.05), and raised the level of SOD (P<0.05) significantly in therapy group. Otherwise, the pyramidal cells remained organized order, with only a few cells degenerated occasionally in rats of therapy group. Conclusion The compound has SOD like activity. It can scavenge the free radical and inhibit peroxidation of lipid, so it can prevent brain cells from the damage.

Objective To understand acute rejection differences between untreated recipients and rapmycin-treated recipients in a rat free flap allotransplantation model. Methods Brown groin free flaps were transplanted to Lewis recipients. In the treated group, recipients were treated with rapamycin at the dose of 4 mg/kg every day from day 0 to day 14 after transplantation. In the untreated group, recipients didn't receive any treatment. Allografts were evaluated clinically and histologically. Results Allografts in the treated group showed epidermolysis as sign of rejection.Rejection sign of untreated grafts was ischemic necrosis of whole skin. In histological evaluation, the treated grafts showed "band-like" lymphocytes infiltration in the upper dermis when rejection occurred, while the untreated grafts showed thrombosis in the subdermal vessels. Conclusion The differences between the two groups implied that embolization may be responsible for the rejection of free flap allotransplantation in rat model.

Objective To evaluate the value of CYRTA21-1,CEA and SPECT in diagnosis of bone metastasis in lung cancer.Methods 216 patients with lung cancer were examined by radionuclide bone imaging and serum CYFRA21-1,CEA.20 patients with benign lung disease were also studied.Results 121 of the 216 lung cancer patients were confirmed bone metastasis including 101 multi-metastasis and 20 singu- lar-metastasis. The positive rate was 56.02 %.The serum CYFR21-1,CEA level and positive rate of lung cancer patients with bone metastasis were obviously higher than those of patients without bone metastasis or with benign lung disease.The level of serum CYFR21-1,CEA had significant difference(P

Objective To explore the roles and possible mechanism of calcium-sensing receptor(CaSR) in cell cardiac hypertrophy model using angiotensin Ⅱ (Ang Ⅱ ).Methods The cultured neonatal rat ventricular myocytes were treated with Ang Ⅱ as cell cardiac hypertrophy model.Hypertrophic neonatal rat cardiomyocytes were treated with GdCl3(a specific agonist of CaSR) and/or with Ro318220(a specific inhibitor of PKC pathway).To evaluate the status of cardiac hypertrophy,cell diameter was observed by HE dyeing,and protein content was determined through coomassie brilliant blue protein kit.The intracellular calcium concentration( [ Ca2+]i) was determined by laser scanning confocal microscope.The protein expression of CaSR and PKC pathway were analyzed using Western blotting.Results ①Compared to the control group(0.1263 ± 0.0443),the protein expression of CaSR was increased in Ang Ⅱ group and in GdCl3 group(0.1963 ± 0.0375,0.2778 ± 0.0564,all P＜ 0.05).Moreover,compared with Ang Ⅱ alone,the increase was significant in GdCl3 group(P ＜ 0.05).②Compared to control group(222.70 ± 22.09),AngⅡ group(392.16 ± 36.85) remarkably increased [Ca2+]i(P＜ 0.05),and this increase of [Ca2+]i was further enhanced in GdCl3 group (502.60 ± 44.21) versus Ang Ⅱ group (P ＜ 0.05).③Compared to control group,Ang Ⅱ could induce cardiomyocyte hypertrophy,and GdCl3 enhanced the effect.Moreover,this enhancement was attenuated by Ro318220.④Compared to control group(0.27 ± 0.07,0.69 ± 0.06,0.87 ± 0.04),the protein expression of PKCα,PKCε and PKCδ was increased in Ang Ⅱ group(0.60 ± 0.16,1.02 ± 0.13,1.20 ± 0.18,all P＜ 0.05) and the protein expression of PKCα,PKCε was increased in GdCl3 group(0.82 ± 0.16,1.34 ± 0.12,all P ＜ 0.05).Moreover,compared with Ang Ⅱ group,the protein expression of PKCα,PKCε was obviously increased in GdCl3 group (all P ＜ 0.05);compared with GdCl3 group,the protein expression of PKCα,PKCε(0.41 ± 0.10,0.85 ± 0.14) was obviously decreased in Ro318220 group(all P ＜ 0.05).Conclusions CaSR is involved in cardiac hypertrophy induced by Ang Ⅱ through PKC pathway in cultured neonatal rat cardiomyocytes.

BackgroundWhether the peeling of the inner limiting membrane (ILMP) increase the closure rate of idiopathic macular hole is still in controversy.Some ophthalmologist recommend vitrectomy combined with inner limiting membrane peeling for the treatment of idiopathic macular hole.However,the removal of ILMP is difficult because of its similar appearance to adjacent tissues.Objective This study was to investigate the efficacy of triamcinolone acetonide(TA) and indocyanine green(ICG) double staining-assisted vitrectomy combined with ILM peeling during the surgery.Methods A consecutive case- observational study was designed.The standardized vitrectomy was performed in 25 eye of 23 cases with IMH.During the vitrectomy,TA and ICG were injected into posterior pole vitreous to visualize and assist the ILM peeling.The dying effectiveness was observed,and the closure rate of macular hole,visual acuity,intraocular pressure and complications were evaluated after surgery.Written informed consent was obtained from each patient prior to operation.Results Posterior vitreous cortex and ILM were visible and the residual vitreous and cortex were removed clearly after dying of TA and ICG in all the 25 eyes.During the following-up duration of 3-8 months,the completely anatomical reattachment of the macular area was in 22 eyes ( 88.0％ ) and partially reattachment in 3 eyes( 12.0％ ).The best corrected vision was 0.07-0.60 in all of the operated eyes 2 months after surgery.Conclusions TA and ICG- assisted vitrectomy combined with ILM peeling appears to be a safe and effective method for IMH repair.

Objective To investigate the expression difference of vascular endothelial growth factor (VEGF)between lung cancer and lung benign disease,and its relation to clinical characteristics and prognosis of non-small cell lung cancer(NSCLC),and to research the possible role of VEGF in the growth of lung can- cer.Methods The expression of VEGF and microvascular density(MVD)were detected in 60 lung cancer tissues and 30 lung benign disease tissues after operation by immunohistochemical method and put up statisti- cal analysis.Results The positive rate of VEGF expression(63%)and MVD(45.13?10.27)in lung cancer tissues were both higher than those in lung benign disease tissues(27%,33?6.49)(P

OBJECTIVETo study the effect of blood activating water relieving method (BAWRM) on heart functions and serum levels of NT-proBNP in patients with heart failure with normal ejection fraction (HFNEF).

METHODSSixty-four HFNEF patients were admitted to our hospital during January 2011 to June 2012. They were randomly assigned to the treatment group (32 cases) and the control group (32 cases). Patients in the control group received routine Western medical treatment, while those in the treatment group additionally took Chinese medical recipes for activating blood circulation and relieving water retention. Changes of Chinese medical syndromes, E/E', serum NT-proBNP contents were observed between the two groups.

RESULTSCompared with before treatment, their Chinese medical syndromes and E/E' were significantly improved, and serum NT-proBNP contents decreased in the two groups (P < 0.05). Compared with the control group, Chinese medical syndromes, E/E', serum NT-proBNP contents obviously decreased in the treatment group, showing statistical difference (P < 0.05).

CONCLUSIONBAWRM was an effective way to improve the diastolic function of HFNEF patients and lower the serum level of NT-proBNP with confirmative efficacy.

Aged ; Female ; Heart Failure ; blood ; drug therapy ; physiopathology ; Humans ; Male ; Medicine, Chinese Traditional ; methods ; Middle Aged ; Natriuretic Peptide, Brain ; blood ; Peptide Fragments ; blood ; Stroke Volume

Objective To report 11 cases with the tissue defects of their upper or lower limbs repaired with the anastomsed medial sural artery perforator flaps.Methods The free medial sural artery perforator flaps,with length of 8 cm to 15 cm and width of 6 cm to 14 cm were used for tissue defect reconstruction of the distal upper or lower limbs in 11 cases,including 6 females and 5 males.The flap was harvested from the ho- mo-lateral calf,confined between the posterior-medial edge of the tibia and the middle line of the calf and a- bove the distal half part of the medial sural muscle,with a same axis of this muscle.Results Ten cases survived very well,which was relatively thin,and the donor site can be acceptable.One case resulted in a complete flap necrotized and covered with a split skin graft.No obvious motor function defect was observed of the donor leg.Conclusion The anastomsed medial sural artery perforator flap is alternative donor flap for the upper or lower limb tissue defect repair,especially for the defect in the hand or foot.