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 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

OBJECTIVEThe novel influenza A (H1N1) virus firstly detected in April 2009 in Mexico rapidly spread to many countries including the United States and Canada where humans were infected with the H1N1 virus and deaths were reported. The pandemic virus strain had never been detected in specimen of human beings and swine. It was so highly contagious and widely spread that threatened life of humans globally. This study aimed to analyze clinical data of hospitalized children patients with 2009 novel H1N1 influenza A virus infection confirmed by etiologic tests, and compared with that of seasonal influenza A.

METHODClinical manifestations, laboratory and therapy data from the hospitalized children were collected by designed case report form and analyzed. All patients were enrolled from Capital Institute of Pediatrics from January 2003 to 2010. There were 152 cases in seasonal influenza A group, which was composed of 100 boys and 52 girls. Other 93 boys and 86 girls formed 2009 novel influenza A group.

RESULTInfluenza A was dominate from 2003 to 2008 and the peak season was December and January, while the peak hospitalized time of 2009 novel H1N1 influenza was from November 2009 to January 2010. The median age of seasonal influenza group was 35 months, which was lower than that of novel influenza group (Z = -6.702, P<0.01). Besides, 80.9% of the patients in seasonal influenza group were infants, while the novel influenza A group was mainly composed of infants and pre-school children (chi2 = 40.725, P<0.01). The cases of both groups had influenza-like symptoms at onset and the most common presentations were fever and cough. The duration of fever was much longer in 2009 novel influenza group (Z = -7.173, P<0.01). Patients in two groups nearly had the same symptoms except cough was more frequently presented by novel influenza A group cases (chi2 = 4.109, P<0.05). In laboratory examination, the novel influenza group had more cases with abnormality in blood platelet, CRP, ALT, and CK-MB than that of seasonal influenza group (chi2 = 7.562, 17.245, 4.398, 6.217, P<0.01). Patients in novel influenza A group had more changes in electrocardiogram (chi2 = 24.461, P<0.01). More patients had common underlying medical condition in novel influenza groups than those in seasonal influenza group (chi2 = 12.553, P<0.01). Furthermore, the groups had different age distribution in underlying medical diseases (chi2 = 7.231, P<0.05). Children with 2009 novel H1N1 virus infection tended to catch pneumonia (chi2 = 8.661, P<0.01) and became the severe cases (chi2 = 10.595, P<0.01). They had much higher ICU admission rate (chi2 = 12.873, P<0.01) and longer hospital stay (Z = -2.764, P<0.01).

CONCLUSIONAs a new variant of influenza virus A, 2009 novel H1N1 influenza A had stronger pathogenicity. Children with underlying medical conditions had the high risk to be infected and developed severe manifestations.

Adolescent ; Child ; Child, Hospitalized ; Child, Preschool ; China ; epidemiology ; Female ; Humans ; Infant ; Infant, Newborn ; Influenza A Virus, H1N1 Subtype ; Influenza A virus ; Influenza, Human ; epidemiology ; virology ; Male

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

BACKGROUNDProteasome subunits (PSMB) and transporter associated with antigen processing (TAP) loci are located in the human leukocyte antigen (HLA) Class II region play important roles in immune response and protein degradation in neurodegenerative diseases. This study aimed to explore the association between single nucleotide polymorphisms (SNPs) of PSMB and TAP and Parkinson's disease (PD).

METHODSA case-control study was conducted by genotyping SNPs in PSMB8, PSMB9, TAP1, and TAP2 genes in the Chinese population. Subjects included 542 sporadic patients with PD and 674 healthy controls. Nine identified SNPs in PSMB8, PSMB9, TAP1, and TAP2 were genotyped through SNaPshot testing.

RESULTSThe stratified analysis of rs17587 was specially performed on gender. Data revealed that female patients carry a higher frequency of rs17587-G/G versus (A/A + G/A) compared with controls. But there was no significant difference with respect to the genotypic frequencies of the SNPs in PSMB8, TAP1, and TAP2 loci in PD patients.

CONCLUSIONChinese females carrying the rs17587-G/G genotype in PSMB9 may increase a higher risk for PD, but no linkage was found between other SNPs in HLA Class II region and PD.

ATP-Binding Cassette Sub-Family B Member 2 ; genetics ; ATP-Binding Cassette, Sub-Family B, Member 3 ; genetics ; Adult ; Aged ; Antigen Presentation ; Case-Control Studies ; Cysteine Endopeptidases ; genetics ; Female ; Humans ; Male ; Middle Aged ; Parkinson Disease ; genetics ; immunology ; Polymorphism, Single Nucleotide ; Proteasome Endopeptidase Complex ; genetics

OBJECTIVETo establish a method for amplifying specific 16S rDNA fragment of algae related with drowning and test its value in drowning diagnosis.

METHODSThirty-five rabbits were randomly divided into 3 the drowning group (n=15), postmortem water immersion group (n=15, subjected to air embolism before seawater immersion), and control group(n=5, with air embolism only). Twenty samples of the liver tissues from human corpses found in water were also used, including 14 diatom-positive and 6 diatom-negative samples identified by microwave digestion-vacuum filtration-automated scanning electron microscopy (MD-VF-Auto SEM). Seven known species of algae served as the control algae (Melosira sp, Nitzschia sp, Synedra sp, Navicula sp, Microcystis sp, Cyclotella meneghiniana, and Chlorella sp). The total DNA was extracted from the tissues and algae to amplify the specific fragment of algae followed by 8% polyacrylamide gelelectrophoresis and sliver-staining.

RESULTSIn the drowning group, algae was detected in the lungs (100%), liver (86%), and kidney (86%); algae was detected in the lungs in 2 rabbits in the postmortem group (13%) and none in the control group. The positivity rates of algae were significantly higher in the drowning group than in the postmortem group (P<0.05). Of the 20 tissue samples from human corps found in water, 15 were found positive for algae, including sample that had been identified as diatom-negative by MD-VF-Auto SEM. All the 7 control algae samples yielded positive results in PCR.

CONCLUSIONSThe PCR-based method has a high sensitivity in algae detection for drowning diagnosis and allows simultaneous detection of multiple algae species related with drowning.

Animals ; Autopsy ; Cadaver ; DNA, Ribosomal ; isolation & purification ; Diatoms ; genetics ; Drowning ; diagnosis ; Electrophoresis, Polyacrylamide Gel ; Humans ; Kidney ; Liver ; Lung ; Microscopy, Electron, Scanning ; Polymerase Chain Reaction ; RNA, Ribosomal, 16S ; isolation & purification ; Rabbits

OBJECTIVETo assess the efficiency of eluting stent coated with arsenic trioxide (As(2)O(3)) suspended in poly-L-lactic acid (PLLA) to prevent in-stent restenosis in rabbits.

METHODSForty-five male New Zealand white rabbits were assigned to three groups (n = 15 for each group) at random: uncoated stents, stents coated with PLLA or stents coated with As(2)O(3) in PLLA. Animals were euthanized 28 days after stent implantation into the iliac arteries of rabbits. Neointimal thicknesses and apoptosis of vascular smooth muscle cell (VSMC) were measured. Stents coated with As(2)O(3) in PLLA were implanted in another 48 male New Zealand white rabbits, As(2)O(3) concentrations in serum and arterial tissue at implantation site were measured at 2 h and 1, 3, 7, 14, 28 days after As(2)O(3) eluting stent implantation (n = 8 for each time point).

RESULTSNeointimal hyperplasia was significantly reduced 51% and 31% and apoptosis significantly increased (21.0 +/- 3.3; 6.2 +/- 1.9(*); 5.3 +/- 2.1(*), (*)P < 0.01 vs. As(2)O(3) eluting stent) with As(2)O(3) eluting stent, versus PLLA-coated stents and uncoated stents. As(2)O(3) concentrations in arterial tissue at implantation site were 18.6 +/- 9.1 (ng/mg) at 1 day and 0.3 +/- 0.1 (ng/mg) at 28 days after stent implantation.

CONCLUSIONSAs(2)O(3) coated stents released As(2)O(3) to local tissue for at least 28 days, suppressed neointimal hyperplasia in rabbit iliac arteries and increased local VSMC apoptosis might be one of the mechanisms for inhibiting restenosis by As(2)O(3) coated stents.

Animals ; Apoptosis ; drug effects ; Arsenicals ; administration & dosage ; Coronary Restenosis ; prevention & control ; Drug-Eluting Stents ; Iliac Artery ; Male ; Muscle, Smooth, Vascular ; cytology ; Oxides ; administration & dosage ; Rabbits ; Random Allocation

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