OBJECTIVETo analyze the clinical and laboratory characteristics of Gitelman syndrome.

METHODSSeventeen patients with Gitelman syndrome (male/female: 11/6) were analyzed for their clinical symptoms, laboratory test results, imaging findings, treatments and outcomes.

RESULTSFifteen of the 17 patients presented with varying degrees of lower limb weakness, and 8 experienced flaccid paralysis. The laboratory tests showed hypokalemia (17/17), hypomagnesemia (17/17) and hypocalcemia (17/17). Blood renin activity (17/17), angiotensin II (14/17) and aldosterone levels (7/17) were significantly higher in the patients than in normal subjects. The symptoms were relieved by potassium alone or in combination with indomethacin, spironolactone and other potassium magnesium asparaginate, but the serum potassium and magnesium failed to recover the normal levels after the treatments.

CONCLUSIONThe primary clinical manifestations of Gitelman syndrome are lower extremity weakness with hypokalemia and hypomagnesemia. Combined drug therapies including potassium, magnesium, aldosterone antagonists and other drugs are recommended. The prognosis of the patients is favorable.

Adolescent ; Adult ; Child ; Female ; Gitelman Syndrome ; diagnosis ; drug therapy ; Humans ; Indomethacin ; therapeutic use ; Male ; Middle Aged ; Potassium Chloride ; therapeutic use ; Potassium Magnesium Aspartate ; therapeutic use ; Retrospective Studies ; Spironolactone ; therapeutic use ; Young Adult

Adolescent ; Bronchial Neoplasms ; diagnostic imaging ; pathology ; surgery ; Bronchoscopy ; Hemangioma ; diagnostic imaging ; pathology ; surgery ; Humans ; Male ; Tomography, X-Ray Computed

OBJECTIVETo explore the epidemiological status and risk factors of hyperuricemia in rural area of the Three Gorges.

METHODSA cross-sectional survey was carried out in rural area of Yiling District, Yichang City, which was located north-west bank of Xiling Gorge in 2007. A standard structure questionnaire was used to collect demographic data, social-economic status and life-style features. Fasting venous blood was collected and serum uric acid (SUA) was determined. Hyperuricemia was defined as SUA levels ≥ 417 µ mol/L (70 mg/L) in men and ≥ 357 µmol/L (60 mg/L) in women. Multiple logistic regression analysis was used to analysed the risk factors of hyperuricemia.

RESULTSA total of 9354 participants aged 35 and above were included, 19.9% (1866/9354) participants were the Three Gorges migrants. Serum uric acid level in men was significantly higher than that in women [(285.1 ± 80.2) µmol/L vs. (210.3 ± 65.0) µmol/L,P < 0.01].Serum uric acid level increased significantly in both genders in proportion to increase of age, and was higher in men than in women in all age groups (all P < 0.01). The age-adjusted prevalence was significantly higher in men than in women (5.6% vs. 3.3%, P < 0.01), and was also higher in men aged 35-44 and aged 45-54 than in women (both P < 0.01). There was no significance in prevalence of hyperuricemia in both men and women aged 55-64 and aged ≥ 65. After adjusting age, gender, educational level, migration and occupation, the multiple logistic regression analysis showed that the prevalence of hyperuricemia was higher in alcohol drinking participants than that of non-alcohol drinking participants (OR = 2.06, 95%CI:1.59-2.67, P < 0.01), and in participants used to consume less green vegetables and fruits than in participants consuming more green vegetables and fruits (OR = 1.77, 95% CI:1.27-2.47, P < 0.01).

CONCLUSIONSThe prevalence of hyperuricemia is relatively low in rural area of the Three Gorges.Alcohol drinking and low intake of green vegetables and fruits are the risk factors of hyperuricemia in this population.

Adult ; Aged ; China ; epidemiology ; Cross-Sectional Studies ; Female ; Humans ; Hyperuricemia ; epidemiology ; Logistic Models ; Male ; Middle Aged ; Risk Factors

OBJECTIVETo investigate HIV-1 co-receptor usage in patients experienced anti-retroviral therapy (ART) in Anhui and Henan province of China.

METHODSA total of 45 HIV-1 infected individuals who have experienced ART and 109 un-experienced ART patients from Anhui and Henan province, which were called as treatment group and treatment-negative group, were selected as study subjects. HIV-1 strains were isolated from peripheral blood mononuclear cells of whole blood from patients. HIV-1 p24 in the culture supernatant was measured using a commercial enzyme-linked immunosorbent assay (ELISA) kit. HIV-1 co-receptor usage was identified using Ghost cell lines expressing CD4 and the chemokine receptor CCR5 or CXCR4.

RESULTSAmong 45 HIV strains from the treatment group, 22 (48.9%) strains used CCR5 as a co-receptor (R5 tropic strain), 21 (46.7%) strains used CXCR4/CCR5 as a co-receptor (X4/R5 duel tropic strain), and 2 (4.4%) used only CXCR4 as a co-receptor (X4 tropic strain). In 109 strains from treatment-negative group, 96 (88.1%) strains used CCR5 as a co-receptor (R5 tropic strain), 13 (11.9%) strains used CCR5/CXCR4 as a co-receptor use (X4/R5 strain). A significant difference was found between two groups in X4 co-receptor usages (χ(2) = 27.30, P < 0.05). Furthermore, after treated with AZT + DDI + NVP, the HIV-1 CXC4/CCR5 utilization was 59.09% (13/22), meanwhile after treated with D4T + DDI + NVP, the HIV-1 CXC4/CCR5 utilization was 43.48% (10/23), which the difference was not statistical significant (χ(2) = 1.10, P = 0.30).

CONCLUSIONHIV-1 CXCR4/CCR5 co-receptor utilization was higher in ART patients than treatment-negative patients.

Acquired Immunodeficiency Syndrome ; drug therapy ; metabolism ; Adult ; Aged ; Antiviral Agents ; therapeutic use ; Cells, Cultured ; Female ; HIV-1 ; isolation & purification ; Humans ; Male ; Middle Aged ; Receptors, CCR5 ; metabolism ; Receptors, CXCR4 ; metabolism ; Receptors, HIV ; metabolism

OBJECTIVETo investigate the mechanism of liraglutide-mediated protection against nonalcoholic fatty liver disease (NAFLD) using aApoE knockout (KO) mouse with high-fat diet (HFD) and Acrp30 knockdown.

METHODSFifty-six male ApoE KO mice were divided into the following six modeling and experimental groups:regular chow fed (ApoE KO, n=10), HFD fed (HF, n=10), HFD+Adenovirus (Ad)-small hairpin (sh) Acrp30 (Ad-shAcrp30, n=10), HFD+Ad-shGreen Fluorescent Protein (GFP) (Ad-shGFP, n=6), HFD+Ad-shAcrp30+liraglutide (liraglutide, n=10), and HFD+Ad-shAcrp30+saline (saline, n=10). Weight-matched C57BL/6 mice on the regular chow diet were used as the control group (WT control, n=10).All mice were fed their assigned diet for 16 weeks.The Ad-shGFP or Ad-shAcrp30 was injected by tail vein at the end of 14 and 15 weeks.Mice in the liraglutide group received 1 mg/kg of the drug, twice daily, intraperitoneally for a total of 8 weeks (from the 9th to 16th week).Fasting blood samples were collected for testing levels of fasting plasma glucose (FPG), triglycerides (TGs), total cholesterol (TC), free fatty acid (FFA), alanine aminotransferase (ALT), Acrp30 and insulin.Liver tissue was procured for histological examination.Expression of mRNA was detected by real-time RT-PC and of protein was detected by western blot analysis.

RESULTSThe Ad-shAcrp30 treated mice had reduced expression of Acrp30 at both the mRNA and protein levels in adipose tissues and plasma, as compared with the AdshGFP treated mice (all P < 0.01).Compared to the WT and ApoE KO groups, the HF group showed higher levels of FPG, FFA, TGs and TC (all P < 0.01); furthermore, the Ad-shAcrp30 treatment compounded these changes.The Ad-shAcrp30 treated group had markedly higher hepatic TC and TGs than the HF group (P < 0.01 and P < 0.05).Oil Red O staining showed that there was more lipid droplets in the liver tissue of the Ad-shAcrp30 treated group than in that of the HF group (P < 0.01), and hematoxylin-eosin staining confirmed these results.Liraglutide treatment prevented the increase in body weight, FPG, FFA, TGs, TC and ALT levels, as compared to the saline controls (all P < 0.01), but the plasma Acrp30 levels and the Acrp30 mRNA and protein expression in adipose tissues were elevated (all P < 0.01).Oil-Red O staining indicated that the liraglutide group had a significantly lower hepatic lipid content than the saline group, and total hepatic TG and TC were reduced in the former group (P < 0.01 and P < 0.05).The liraglutide treatment significantly attenuated the mRNA expression of ACC and FAS (both P < 0.01) but increased AMPK phosphorylation (P < 0.01).

CONCLUSIONAdministration of liraglutide prevented the development of HFD-and hypoadiponectinemia-induced metabolic disturbance and accumulation of hepatic lipids in this mouse model system of NAFLD.

AMP-Activated Protein Kinases ; metabolism ; Adiponectin ; deficiency ; metabolism ; Adipose Tissue ; Alanine Transaminase ; Animals ; Apolipoproteins E ; deficiency ; metabolism ; Cholesterol ; Diet, High-Fat ; Disease Models, Animal ; Glucagon-Like Peptide 1 ; analogs & derivatives ; Insulin ; Liraglutide ; Male ; Metabolism, Inborn Errors ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Non-alcoholic Fatty Liver Disease ; metabolism ; Protective Agents ; RNA, Messenger ; Triglycerides

Pentraxin 3 (PTX3) was identified as a marker of the inflammatory response and overexpressed in various tissues and cells related to cardiovascular disease. Honokiol, an active component isolated from the Chinese medicinal herb Magnolia officinalis, was shown to have a variety of pharmacological activities. In the present study, we aimed to investigate the effects of honokiol on palmitic acid (PA)-induced dysfunction of human umbilical vein endothelial cells (HUVECs) and to elucidate potential regulatory mechanisms in this atherosclerotic cell model. Our results showed that PA significantly accelerated the expression of PTX3 in HUVECs through the IkappaB kinase (IKK)/IkappaB/nuclear factor-kappaB (NF-kappaB) pathway, reduced cell viability, induced cell apoptosis and triggered the inflammatory response. Knockdown of PTX3 supported cell growth and prevented apoptosis by blocking PA-inducted nitric oxide (NO) overproduction. Honokiol significantly suppressed the overexpression of PTX3 in PA-inducted HUVECs by inhibiting IkappaB phosphorylation and the expression of two NF-kappaB subunits (p50 and p65) in the IKK/IkappaB/NF-kappaB signaling pathway. Furthermore, honokiol reduced endothelial cell injury and apoptosis by regulating the expression of inducible NO synthase and endothelial NO synthase, as well as the generation of NO. Honokiol showed an anti-inflammatory effect in PA-inducted HUVECs by significantly inhibiting the generation of interleukin-6 (IL-6), IL-8 and monocyte chemoattractant protein-1. In summary, honokiol repaired endothelial dysfunction by suppressing PTX3 overexpression in an atherosclerotic cell model. PTX3 may be a potential therapeutic target for atherosclerosis.
Apoptosis/drug effects ; Atherosclerosis/chemically induced/*drug therapy/immunology/pathology ; Biphenyl Compounds/chemistry/isolation & purification/*pharmacology ; C-Reactive Protein/*genetics/immunology ; Down-Regulation/drug effects ; Drugs, Chinese Herbal/chemistry/isolation & purification/*pharmacology ; Human Umbilical Vein Endothelial Cells ; Humans ; I-kappa B Kinase/*immunology ; Lignans/chemistry/isolation & purification/*pharmacology ; Magnolia/chemistry ; Palmitic Acid ; Protein-Serine-Threonine Kinases/*immunology ; Serum Amyloid P-Component/*genetics/immunology ; Signal Transduction/drug effects

OBJECTIVETo establish a HPLC fingerprint for quantitative analysis of the active constituents of jizhi syrup.

METHODHPLC analysis was performed on a Zorbax Sb C18 column (4.6 mm x 250 mm, 5 microm), the mobile phase in gradient elution was composed of (A) 1% acetic acid solution (including 0.2% triethylamine) and (B) acetonitrile, at a flow rate of 1.0 mL x min(-1). The column temperature was 30 degrees C and the wavelength was 280 nm.

RESULT21 common peaks were tested in 10 batches of samples. Compared with the standard fingerprint, the similarity of each sample was greater than 0.99. At the same time, protocatechuic acid, protocatechu aldehyde, chlorogenic acid, caffeic acid, naringin and neohesperidin were found in all samples.

CONCLUSIONThe established method can be used for the quality control of jizhi syrup.

Antitussive Agents ; chemistry ; Benzaldehydes ; analysis ; Catechols ; analysis ; Chlorogenic Acid ; analysis ; Chromatography, High Pressure Liquid ; methods ; Drug Combinations ; Drugs, Chinese Herbal ; chemistry ; isolation & purification ; Flavanones ; analysis ; Hydroxybenzoates ; analysis ; Plants, Medicinal ; chemistry ; Reproducibility of Results

Objective The epidemiological characteristics of congenital heart disease(CHD)among Tibetan children whose age ranged from 4 to 18 at different altitude were investigated in Qinghai province.Methods 32 578 Tibetan children living at 2535 m,3600 m and 4200 m were surveyed with the following 3 steps:prescreened,counterchecked and diagnosed with color Doppler.The entity distribution was then analysed and the age and gender were compared respectively.Resuits 235 CHD cases were identified.The total morbidity was 7.21‰.CHD morbidity was rising with the increase of altitude with 5.45‰ at 2535 m,6.80‰ at 3600 m and 9.79‰ at 4200 m respectively.There were significant static differences between 4200 m and the others with χ2=7.002(P＜0.01)to 2535 m and χ2=5.540(P＜0.05)to 3600 m.However,there was no statistical difference between 2535 m altitude and 3600 m altitude.The morbidity in different age had no statistical difference at 2535 m altitude but statistically increased with the increase of age at 3600 m and 4200 m.The total ratio of 16-18 age was significantly higher than other age periods with χ2=10.79(P＜0.005)to 4-7 age period and with χ2=5.60(P＜0.05)to 8-12 age period.The atrial septal defect(ASD)morbidity rates in three places was 39.1%followed by ventricular septal defect(VSD)with 32.8%and patent duetus arteriosus(PDA)with 24.7%.However,the constitute of CHD was different in different altitudes that VSD with 43.5%at 2535 m.ASD with 42.8%at 3600 m and PDA with 50.8%at 4200 m which was the highest morbidity.Conclusion Morbidity.constitutes and difference in gender and age were related to altitude.

Aim To investigate pro-angiogenic effect of SEHM formula on HUVEC cells in vitro and Zebrafish in vivo and the related action mechanism .Methods VEGFR tyrosine kinase inhibitor II ( VRI)-induced ze-brafish intersegmental vessels ( ISVs ) damaged model was established to observe the protective effect of SE-HM formula on ISVs under fluorescence microscope . The pro-angiogenic effect on subintestinal vessels ( SIVs ) of water decoction of SEHM formula in ze-brafish was observed and the mRNA level of VEGFRs , including flt-1, kdr, kdrl, was measured by real-time PCR.The experimental models of the HUVEC cells were set up and the toxicity and promoting proliferation effect of water decoction of SEHM formula in HUVEC cells were assessed by cell viability assay (MTT), and then the levels of Akt, p38, p-p38, p44/42, p-p44/42, VEGFR-1 were detected by Western blot at 6, 12 and 24 h.Results SEHM formula treatment groups could significantly protect VRI-induced zebrafish ISVs loss(P<0.01) and presented pro-angiogenic effect on zebrafish SIVs obviously(P<0.01).The mRNA level of VEGFRs, including flt-1, kdr, kdrl.was up-regula-ted by SEHM formula treatment group significantly (P<0.05) compared with VRI group.Compared with control group , and SEHM formula treatment group could apparently promote proliferation in HUVEC cells and up-regulate the level of Akt, p38, p-p38, p44/42, p-p44/42, VEGFR-1.Conclusions Water de-coction of SEHM formula could present pro-angiogenic effect on SIVs in zebrafish and promote proliferation of HUVEC cells significantly , and its action mechanism may be related to up-regulating the expression of VEG-FR.

BACKGROUNDAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart anomaly. We aimed to illustrate the clinical features and long-term prognosis of patients with ALCAPA.

METHODSTwenty three patients (13 males and 10 females, ages ranging from 2.5 months to 65 years) identified as ALCAPA in Beijing Anzhen Hospital from April 1984 to June 2009 were divided into two groups, based on the age of onset: group 1 (≤ 12 months, n = 16) and group 2 (> 12 months, n = 7).

RESULTSFifty six point three percent of patients in group 1 had been misdiagnosed as endocardial fibroelastosis (9/16), 18.8% as dilated cardiomyopathy (3/16) and 6.3% as myocardial infarction (1/16). Patients in group 2 were usually diagnosed as coronary heart disease, myocarditis, or patent ductus arteriosus. Electrocardiography in group 1 revealed abnormal Q waves with T wave inversion in leads I, avL, V(4)-V(6), especially in lead avL (deep and wide Q wave); but no specific manifestations in group 2. A higher percentage of patients in group 1 had cardiomegaly on chest radiograph (86.7% vs. 33.3%, P = 0.031), while pulmonary artery protrusion was more common in group 2 (26.7% vs. 83.3%, P = 0.046). Lower left ventricular ejection fraction (LVEF) was present in group 1 than in group 2 ((48.5 ± 11.5)% vs. (65.0 ± 6.1)%, P < 0.001). Apical ventricular aneurysm (62.5% vs. 0%, P = 0.007), enhanced echogenicity of papillary muscles (87.5% vs. 28.6%, P = 0.011) and endocardial thickening (93.8% vs. 14.3%, P < 0.001) were more frequent in group 1 than in group 2. The ratio of the proximal right coronary artery (RCA) diameter to the aortic root diameter exceeded 0.14 in all cases, more prominent in group 2 (0.26 ± 0.05 vs. 0.33 ± 0.03, P = 0.009). Increased coronary artery collaterals within the interventricular septum were detected in 18 patients (78.3%) by Doppler imaging. Twenty one patients underwent cardiac surgery, including left coronary artery (LCA) ligation (1/21), LCA ligation plus coronary artery bypass grafting (1/21), Takeuchi operation (7/21), and LCA reimplantation surgery (12/21). Four patients underwent concomitant mitral valve repair and one received mitral valve replacement. Aneurysm resection was performed in 3 cases. Six patients died in hospital after surgery, and the rest of the cohort had no overt symptoms during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed or disappeared, and the LVEF and left ventricle size returned to normal range with alleviation of mitral insufficiency.

CONCLUSIONSThe accurate diagnosis of ALCAPA can be made with serial diagnostic methods. ALCAPA can be successfully treated with several types of cardiac surgery, and surgeries of establishing two-coronary-artery circulation are the preferred operations nowadays, with good long-term prognosis.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Coronary Vessel Anomalies ; diagnosis ; mortality ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Middle Aged ; Prognosis ; Pulmonary Artery ; abnormalities