The hereditary spastic paraplegias (HSPs or SPGs) are clinically and genetically highly heterogeneous neurodegenerative disorders mainly characterized by progressive spasticity and weakness in the lower limbs. The inheritance mode includes autosomal dominant(AD-HSP), autosomal recessive(AR-HSP) and X-linked recessive(XR-HSP). Thirty-five loci have been mapped with 17 disease-associated genes identified. SPG4 and SPG7 are the common subtypes in the AD-HSP and AR-HSP, respectively. The authors briefly review the function of spastin (SPG4) and paraplegin (SPG7), both of which belong to AAA ATPases family, and the recent progress of the study on the pathogenesis of HSPs.
Adenosine Triphosphatases ; genetics ; Age of Onset ; Chromosome Mapping ; DNA Mutational Analysis ; Genetic Heterogeneity ; Genotype ; Humans ; Phenotype ; Spastic Paraplegia, Hereditary ; genetics

Fifty-six patients with Graves' ophthalmopathy(GO)were treated with antithyroid drug and oral prednisone for three months,TSH receptor antibody(TRAb)level was reduced,GO activity and severity of some patients were ameliorated but still positively associated with TRAb.It suggests that TRAb not only triggers off GO but also plays a possible role in the maintenance of the autoimmune process in GO.

BACKGROUNDMost of the studies on traditional Chinese medicine (TCM) 'spleen' deficiency syndrome in the recent 30 years were conducted only on the basis of single functional index, neglecting the study on the pathophysiologic internal relationship between spleen deficiency syndrome and gastric diseases in modern medicine. But it was at the subcellular molecular biological level that we explored the pathophysiologic basis of classification of spleen deficiency in chronic gastritis by detecting the bioactive substances in gastric mucosa nuclei and mitochondria.

METHODSBy means of optical microscope, scanning electron microscope (SEM), transmission electron microscopy (TEM) and histochemical staining, we conducted histopathological, subcellular ultrastructural analysis and nuclei and mitochondrial ultrastructural analysis of gastric mucosa of 188 spleen deficiency patients and of 42 voluntary blood donors. At the same time, bioactive substances were measured by means of X-ray energy dispersive analysis system (EDAX) image analysis system, radioimmunoassay method and chemiluminescence method.

RESULTSThe content of cAMP, superoxide dismutase (SOD), Zn and Cu in gastric mucosa, and the content of Zn and Cu in mitochondria decreased progressively in order of groups: healthy control (HC), spleen Qi deficiency without organic lesion (F-SQD), spleen Yang deficiency without organic lesion (F-SyangD), disease without symptoms group, spleen Qi deficiency with organic lesion (G-SQD), spleen Yang deficiency with organic lesion (G-SyangD), spleen Yin deficiency (SyinD) and spleen deficiency with Qi stagnation (SDQS), chronic spleen deficiency gastritis (CSG) and chronic atrophic gastritis (CAG); decreased in order of HC, intestinal metaplasia (IM)Ia, IMIb, IMIIa and IMIIb, P < 0.05. The content of DNA, Zn and Cu in nuclei progressively increased in order mentioned above, P < 0.05.

CONCLUSIONSThe quantitative changes of gastric mucosal cAMP, SOD, Zn, Cu, of mitochondrial Zn, Cu and of nuclear DNA, Zn and Cu are not only the substance base on which the lesion of gastric mucosa tissue structure occurs, but also the substance base on which spleen deficiency is classified. G-SQD and G-SyangD were more likely to be found in low-grade or middle-grade CSG and CAG, while SyinD and SDQS in middle-grade or high-grade CSG, CAG and IMIIb.

Adult ; Aged ; Chronic Disease ; Cyclic AMP ; analysis ; Female ; Gastric Mucosa ; pathology ; ultrastructure ; Gastritis ; metabolism ; pathology ; Humans ; Lipid Peroxides ; blood ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Splenic Diseases ; classification ; Superoxide Dismutase ; analysis

BACKGROUNDSpleen in Traditional Chinese Medicine (TCM) is not actually the spleen in the anatomic sense designated in western medicine because its functions basically belong to the physiological category of digestive system in modern medicine, and it represents a macroscopic concept of digestion, absorption and nutrition metabolism. Spleen deficiency syndrome refers to the clinical phenomena such as hypofunction of digestion, absorption and nutrition metabolism. By integrating TCM with modern medicine, this paper is intended to explore the pathological basis of classification of spleen deficiency in chronic gastritis.

METHODBy means of optical microscope, scanning electron microscope (SEM), transmission electron microscope (TEM) and histochemical staining, we conducted histopathological and subcellular ultrastructural (nuclei and mitochondrial) analysis of gastric mucosa of 188 patients of spleen deficiency, and that of 42 voluntary blood donors without clinical symptoms.

RESULTSThe gastric mucosa of patients with spleen Qi deficiency (SQD) and spleen yang deficiency (SyangD) could either be affected by organic lesion (type G-occurring on the basis of chronic superficial gastritis (CSG), chronic atrophic gastritis (CAG)) or unaffected (type F-chiefly belonging to functional indigestion); spleen yin deficiency (SyinD) and spleen deficiency with Qi stagnation (SDQS) both occurred on the basis of CSG and CAG; and the degree of mucosa inflammatory cells infiltration, the degree of decrease in glands propria, and the incidence of IMIIb in CSG and CAG were more serious than those of G-SQD and G-SyangD, P < 0.05 - 0.01.

CONCLUSIONSpleen deficiency syndrome is likely to occur on the basis of organic lesion of gastric mucosa (disease with symptoms of both CSG or CAG and spleen deficiency symptoms), as well as on the basis of inorganic lesion of gastric mucosa (nondisease with symptoms, which is, despite spleen deficiency symptoms, there is no CSG or CAG). Besides, the clinical phenomenon of disease without symptoms (despite CSG or CAG, there is no spleen deficiency symptoms) occurres because of such factors as genetic diathesis and compensation. The lesion degree of CSG or CAG and the incidence of IMIIb of SyinD and SDQS are more serious than those CSG and CAG of G-SQD and G-SyangD.

Adult ; Chronic Disease ; Digestive System Diseases ; classification ; Epithelial Cells ; ultrastructure ; Female ; Gastric Mucosa ; pathology ; ultrastructure ; Gastritis ; pathology ; Humans ; Male ; Medicine, Chinese Traditional ; Splenic Diseases ; classification

OBJECTIVETo study the physiopathologic basis of Weikangfu Granule (WKFG) in treating precancerosis of gastric mucosa in patients of chronic gastritis with Pi-deficiency syndrome (CG-PDS).

METHODSOne hundred and fifteen patients of CG-PDS who suffered from intestinal metaplasia (IM) and atypical hyperplasia (ATHP) of gastric mucosa, were divided into two groups. The treated group (n = 61) was treated by WKFG with its ingredients modified according to the syndrome type of patients. The control group (n = 54) was treated with Weishu granule. The histopathological and subcellular ultrastructural changes were detected by optical microscope, screening electronic microscope, transmission electronic microscope and histochemical staining; the nuclear and mitochondrial ultrastructure of gastric mucosa were analyzed with energy dispersion X-ray analyser and image analysis system. And the changes of cAMP, lipid peroxide (LPO), superoxide dismutase (SOD) before and after treatment in the treated group were measured and compared with those of the health control group consisting of 15 volunteers.

RESULTSThe symptomatic and pathological therapeutic effect in the treated group were significantly superior to those in the control group (P < 0.05). The contents of Zn, Cu, cAMP, SOD and (3)H-TdR LCT in gastric mucosa of the treated group before treatment were all lower than those of the healthy control group, yet all these indexes markedly increased after treatment, while serum LPO level, which increased before treatment was lowered after treatment. All the changes showed statistical significance (P < 0.05 or P < 0.01).

CONCLUSIONWKFG can reverse IM and ATHP in patients of CG-PDS, and the effect may be realized by way of increasing the level of Zn, Cu, cAMP and SOD in gastric mucosa, promoting cell differentiation, enhancing cellular immunity and reducing oxygen free radicals and lipid peroxidation.

Adult ; Aged ; Antineoplastic Agents ; pharmacology ; therapeutic use ; Chronic Disease ; Copper ; analysis ; Cyclic AMP ; analysis ; Drugs, Chinese Herbal ; therapeutic use ; Gastric Mucosa ; chemistry ; pathology ; ultrastructure ; Gastritis, Atrophic ; pathology ; Humans ; Lipid Peroxides ; analysis ; Male ; Medicine, Chinese Traditional ; Middle Aged ; Precancerous Conditions ; pathology ; Stomach Neoplasms ; pathology ; Superoxide Dismutase ; analysis ; Syndrome ; Yang Deficiency ; complications ; Zinc ; analysis

OBJECTIVETo evaluate the value of adenosine tissue Doppler stress echocardiography on ischemic myocardium.

METHODSRoutine dosage (140 microgxkg(-1)xmin(-1) IV for 6 min) adenosine stress echocardiography was performed on 40 patients with chest pain for diagnosis of coronary artery disease (CAD). The images of left ventricular myocardial motion were acquired by tissue Doppler imaging (TDI) based on traditional 2D stress echocardiography before and 3 min, 6 min after adenosine stress (GE Vivid 7, USA). The myocardial velocity, strain and strain rate in 16 segments were offline measured and analyzed on ECHOPAC software. The results were compared with that of coronary angiography (CAG).

RESULTSCAG identified 18 CAD and 22 non-CAD patients with 159 ischemic segments and 465 non-ischemic segments. Adenosine significantly increased the systolic velocity (Sm), early diastolic velocity (Em), late diastolic velocity (Am), peak systolic strain (Smax), systolic strain rate (SRs), early diastolic strain rate (SRe) and late diastolic strain rate (SRa) both ischemic and non-ischemic segments (all P < 0.05). The baseline Sm and Em in ischemic segments were significant lower than non-ischemic segments [(3.16 +/- 1.20) cm/s vs (4.03 +/- 1.27) cm/s, P < 0.01; (3.75 +/- 1.67) cm/s vs (4.66 +/- 1.70) cm/s, P < 0.05]. At peak stress the differences in Sm and Em were more significant [(3.98 +/- 1.63) cm/s vs (5.07 +/- 1.52) cm/s; (4.51 +/- 2.32) cm/s vs (6.52 +/- 2.56) cm/s; P < 0.01]. The reductions on Smax and Se were more significant in ischemic segments compared those in non-ischemic segments (16.91% +/- 3.35% vs 19.56% +/- 5.47%, P < 0.01 and 9.53% +/- 2.89% vs 13.06% +/- 4.63%, P < 0.001). The biggest area under curve (AUC) in peak stress was seen in Se by ROC curve analysis (AUC = 0.740, with sensitivity 67% and specificity 83%).

CONCLUSIONParameters derived from TDI offer reliable and accurate information on ischemic myocardium during adenosine stress echocardiography.

Adenosine ; Diastole ; Echocardiography, Doppler ; Echocardiography, Stress ; Humans ; Myocardium

OBJECTIVEThe aim of the study is to evaluate the left ventricular (LV) dyssynchrony in chronic heart failure (HF) patients with normal and wide QRS duration.

METHODSTime to peak velocity at peak systolic and early diastolic phase (Ts and Te) were determined in 12 segments of LV by echocardiography (GE Vivid 7) in 54 HF patients (28 with wide and 26 with normal QRS duration) and 15 normal controls to evaluate LV systolic and diastolic dyssynchrony. The risk factors related to LV dyssynchrony were also evaluated.

RESULTSLV end systolic and diastolic volumes were significantly larger and 12 segmental mean Ts and maximal Te difference (Te-diff) were significantly higher in HF patients with wide QRS duration than HF patients with normal QRS duration. Using mean Ts >or= 182 ms as the cut-off value, systolic dyssynchrony was present in 46% HF patients with normal QRS and 71% HF patients with wide QRS. Using Te-diff >or= 79 ms as the cut-off value, diastolic dyssynchrony was seen in 58% HF patients with normal QRS and 89% HF patients with wide QRS. Combined systolic and diastolic dyssynchrony was seen in 31% HF patients with normal QRS and in 64% HF patients with wide QRS. Systolic dyssynchrony was significantly correlated to LV end systolic volume and diastolic dyssynchrony was correlated to end diastolic volume.

CONCLUSIONPercentage of LV dyssynchrony was significantly higher in HF patients with wide QRS, especially in HF patients with increased LV end systolic and diastolic volume.

Adult ; Aged ; Case-Control Studies ; Echocardiography, Doppler, Pulsed ; Female ; Heart Failure ; diagnostic imaging ; physiopathology ; Heart Ventricles ; diagnostic imaging ; physiopathology ; Humans ; Male ; Middle Aged ; Ventricular Dysfunction, Left ; diagnostic imaging ; physiopathology

Objective To delineate the relationship between body mass index(BMI),waist-to-height ratio (WHtR)and hypertension in adolescents.Methods A cross-sectional study was conducted in 3874 adolescents ages 13-18 years.They were classified into four groups according to BMI and WHtR,group A:normal BMI and WHtR

A systemic treatment of granulocyte-colony stimulating factor (G-CSF) is known to improve healings of damaged tissues. However, recent studies suggested local actions of G-CSF on the healing processes of damaged tissues. We investigated the treatment effect of locally injected G-CSF and compared to that of systemically injected G-CSF in a rat model. A wound was created on the rat dorsum and treated either by local injection or by systemic injection of G-CSF. Wound healing rate, deposition of collagen, and gene expression were evaluated. G-CSF receptor (G-CSFR) protein was detected by Western blotting. The wound healing rate in the local injection group was significantly higher than that in the systemic injection group at days 9 and 15; it was also significantly higher than that in the control group at days 3, 9, and 15. The expression of G-CSFR protein in wound tissues was higher than in normal skin tissues. The local injection of G-CSF is more effective than systemic injection of G-CSF in promoting wound healing, which may implicate the local action of G-CSF treatment in wound healing processes.
Animals ; Blotting, Western ; Collagen ; Gene Expression ; Granulocyte Colony-Stimulating Factor ; Models, Animal ; Rats* ; Receptors, Granulocyte Colony-Stimulating Factor ; Skin ; Wound Healing* ; Wounds and Injuries*

Objective To explore the risk factors of cardiovascular disease in adolescents with a family history of hypertension.Methods A cross-sectional study was conducted in 3874 adolescents ages 13-18 years,with normal BP in 3724 people.Based on family history of hypertension (FH),the cohort of adolescents were dichoto- mized as postive family history (FH~+,n=1145) and negative (FH~-,n=2579).Height,weight,waist circum- ference,hip circumference,blood pressure and fasting plasma glucose(FPG),total cholesterol(TC),triglyceride (TG) and high-density lipoprotein cholesterol(HDL-C) were determined.Results FH~+ adolescents had signifi- cantly higher levels of body mass index(BMI),waist circumference,WHR,FPG,TC and LDL-C(P