Objective To study the clinical features of congenital glucose-galactose malabsorption (CGGM),and to improve the understanding of CGGM.Method Clinical manifestations and treatment process of one patient with CGGM in our hospital were retrospectively analyzed.From 1966 to 2016 May,Chinese medical database and PUBMED were searched using Malabsorption syndrome,dehydration,hypernatremia , diarrhea , newborn , carbohydrate metabolism ,andglucose/galactose malabsorption as key words.The clinical features of CGGM reported in literatures were summarized.Result The patient in our hospital was a full-term female infant naturally delivered.The onset of the disease was on the 9th day after birth,and the clinical manifestations included severe diarrhea,severe dehydration,hypernatremia,metabolic acidosis and malnutrition.After intravenous infusion and symptomatic treatment,dehydration,hypernatremia and metabolic acidosis were corrected.However,there was no improvement of diarrhea characterized with watery and acidic stools,and neither was weight gain.Glucose loading test was negative,and fructose loading test was positive.Diarrhea was improved markedly using diagnostic carbohydrate-free formula,so CGGM was diagnosed clinically.SLC5A1 homozygous IVS7-2 A ＞ G mutation was detected which confirmed the diagnosis of CGGM.With carbohydrate-free formula feeding,the body weight of the infant was increased.Followed up for 2 months now,her body length and body weight were at P25 and P22 on growth curve respectively,and no obvious neurological sequela was observed.Our literature review revealed 7 reports including 48 cases of CGGM children.Literature review showed that:most children with CGGM (79.2％) had the onset within 7 days of life;main clinical features included diarrhea (100％),dehydration (100％),and malnutrition (54.2％);22.9％ of patients with carbohydrate-free formula and 27.1％ with fructose matrix formula were fed well;no death was detected,77.1％ had normal weight gain,and 91.7％ had normal development of the nervous system.Conclusion CGGM is rare.The symptoms include severe watery and acidic stools with onset during neonatal period.CGGM is associated with severe complications such as hypertonic dehydration and hypernatremia.The diagnosis is established based upon typical clinical manifestations,sugar loading test and SLC5A1 gene detection.Carbohydrate-free formula feeding is effective.

Objective To investigate the effects of enteral nutrition with galactooligosaccharides (GOS) on serum inflammatory cytokines in rats with severe acute pancreatitis (SAP). Methods SD rats were randomly divided into sham operation control group, SAP with enteral nutrition (EN) group and SAP with EN supplemented with GOS (GOS-EN) group, and each group was divided into 4 d and 7 d subgroups according to the time that animals were sacrificed (n=8 in each subgroup). Rat SAP models were established by injection of 38 g/L sodium taurocholate beneath the pancreatic capsule. The serum amylase, inflammatory cytokines TNF-α, IL-2 and IL-10 were detected. Results At each time point, the levels of serum amylase in all SAP groups were significantly higher than those in sham operation control group (P < 0.01), and the levels in GOS-EN group were significantly lower than those in EN group (P < 0.01). The levels of serum TNF-α and IL-10 in all SAP groups were significantly higher than those in sham operation control group (P < 0.01), while the levels of IL-2 in all SAP groups were significantly lower than those in sham operation control group. The levels of TNF-α in GOS-EN group were significantly lower than those in EN group (P <0.05), while the levels of IL-2 and IL-10 and the ratio of IL-10/TNF-α were significantly higher than those in GOS-EN group (P < 0.05). Conclusion Early EN supplemented with GOS could modulate the balance of pro- and anti-inflammatory response.

The change of yield and contents. of active compositions were studied while the fibrous roots were decayed naturally. HPLC method was used to detect the contents of active composition. The results show that fibrousroots could decrease the production of plant by 38.60% (20 g) and 30.99% (40 g), respectively. Treatment 1 could increase the contents of dihydrotanshinone and cryptotanshinone of Salvia miltiorrhiza f. alba by 26.08% and 22.64%, respectively. Compared with the comparison, treatment 2 decreased the contents of ihydrotanshinone, cryptotanshinone, tanshinone I and tanshinone II(A) of S. miltiorrhiza f. alba by 60.87%, 79.24%, 84.61% and 88.99%, respectively. Meanwhile, the total contents of the liposoluble constituents reduced by 86.27%. The different concentration of fibrousroots could increase the content of salvianolic acid B by 4.98% (20 g) and 23.64% (40 g), respectively. Meanwhile, the content of rosemary acid was increased by 4.98% (20 g) and 23.64% (40 g), respectively. The content of water-soluble constituents positively correlated to the mount of added fibrousroots, and the change was significantly. The result indicted that the decay of fibrousroots has a significant impact on the growth and the content of the active composition of S. miltiorrhiza f. alba under the condition of continuous cropping. Fibrousroots could decrease the content of biomass and liposoluble constituents significantly, which maybe one of the main factors to S. miltiorrhiza f. alba continuous cropping obstacle formation.
Benzofurans ; analysis ; Biomass ; Chromatography, High Pressure Liquid ; Diterpenes, Abietane ; analysis ; Plant Roots ; metabolism ; Salvia miltiorrhiza ; chemistry ; growth & development

Objective:To explore whether sleep quality suffers in patients with mild Alzheimer's disease(AD)and mild cognitive impairment(MCI), and to further investigate the correlation between sleep disorders and cognitive function in these patients.Methods:In this study, 30 mild AD patients, 39 MCI patients and 43 demographically matched healthy controls were enrolled.Sleep quality was assessed by the Pittsburgh sleep quality index(PSQI), and cognitive function was assessed by the mini-mental state examination(MMSE), the Montreal cognitive assessment(MoCA)and a set of neuropsychological scales.The correlation of sleep quality with cognitive function was analyzed for the three groups.Results:Differences were significant in sleep time score[0.0(1.0), 1.0(2.0) vs.1.0(1.0), F=8.18, P=0.02]and daytime function score[1.0(1.0), 1.0(1.0) vs.0.0(1.0), F=8.73, P=0.01]between mild AD, MCI and health control groups.Spearman correlation analysis suggested that scores of sleep disorders were negatively correlated with DSB( r=-0.43, P=0.02)and scores of daytime function were positively correlated with ADL( r=0.39, P=0.03)in patients with mild AD.In addition, scores of sleep quality were negatively correlated with the DSB score( r=-0.40, P=0.01), scores of sleep disorders were positively correlated with ADL( r=0.45, P<0.01), scores of daytime function were negatively correlated with DSF( r=-0.42, P=0.01), DSB( r=-0.62, P<0.01)and VFT-S( r=-0.33, P=0.04), and the total PSQI score was negatively correlated with DSF( r=-0.45, P=0.01)and DSB( r=-0.44, P=0.01)in the MCI group. Conclusions:Patients with mild AD and MCI have longer sleep time and impaired daytime function than healthy people, and sleep quality is correlated with memory, attention and daily living ability in patients with mild AD and MCI.

Objective To analyze the clinical features,laboratory results,treatment and prognosis of children with idiopathic pulmonary hemosiderosis (IPH).Methods The documents of 25 children with IPH,who were hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics from Apr.1992 to Nov.2008 were reviewed.Then some of the patients were followed up.Results The median age of onset was 4 years old (6.5 months to 8 years old).Those patients aged from 3 to 6 years old ranked the first as 48％ (12 cases).The median age at diagnosis was 5.17 years old.The course of desease was between 10 days and 6 years,the median course was 1 year.Cough,pallor,fever and hemoptysis were the common clinical features of IPH patients.The chest radiological patterns for IPH were diverse.The common features of high resolution CT scan in 15 patients included declined transparency and ground glass shadows in 11 cases,cloudy patchy infiltrate in 9 cases,reticular changes in 6 cases,and nodular changes in 2 children.Twentythree cases were once misdiagnosed and 60％ of them were delayed in diagnosis as IPH for more than 1 year.Glucocorticoid therapy was effective in improving symptoms.Some patients suffered from rheumatoid arthritis later in their life.Conclusions The manifestations of IPH in children are nonspecific,therefore it is easily to be misdiagnosed.Combined chest radiographic presentations with repeatedly looking for hemosiderin-laden macrophages in sputum,gastric aspirate or bronchoalveolar lavage fluid are helpful in diagnosis.Glucocorticoid therapy can control the symptoms.Some relationships may exist between IPH and rheumatoid arthritis.

OBJECTIVETo observe the effects of Naoyikang (NYK) on expression of choline acetyltransferase (ChAT) in brain of rats with Alzheimer' s disease (AD).

METHODBilateral infusions of Ibotenic acid (IBO) into nucleus basalis of Meynert (NBM) using hamilton syringe and stereotaxic apparatus were adopted to establish the rat model of AD. After intragastrically administrated with different solution for 28 days, immunohistochemistry and Western-blot were adopted to study the expression of ChAT in frontal cortex of AD rats.

RESULTNYK could improve the morphology and increase the number of ChAT immunoreactive neurons, and significantly promote ChAT protein expression.

CONCLUSIONNYK may be able to increase the synthesis of acetylcholine (ACh) through elevating the expression of ChAT protein, thus improving the level of brain ACh so as to protect central cholinergic neurons.

Alzheimer Disease ; enzymology ; Animals ; Blotting, Western ; Brain ; drug effects ; enzymology ; Choline O-Acetyltransferase ; metabolism ; Disease Models, Animal ; Drugs, Chinese Herbal ; pharmacology ; Gene Expression Regulation ; drug effects ; Immunohistochemistry ; Male ; Rats ; Rats, Sprague-Dawley

The ethyl acetate extract of the flower of Albizia julibrissin was isolated and purified by silica gel, Sephadex LH-20 and MCI GEL CHP-20P column chromatography to yield 29 compounds. Their structures were elucidated as 8-hydroxy-2, 6-dimethyl-2E, 6Z-octadienoic acid (1), 8-O-formyl-2, 6-dimethyl-2E, 6Z-octadienoic acid (la), 8-hydroxy-2, 6-dimethyl-2E, 6E-octadienoic acid (2), 8-O-formyl-2, 6-dimethyl-2E, 6E-octadienoic acid (2a), (2E, 6S)-2, 6-dimethyl-6-O-beta-D-xylpyranosyloxy-2, 7-menthia-folic acid (3), clovan-2beta, 9alpha-diol (4), 2beta-O-formyl-clovan-9alpha-ol (4a), 2beta, 9alpha-O-diformyl-clovan (4b), vomifoliol (5), (6S, 9R)-roseoside (6), vanillin (7), 4-O-ethylgallic acid (8), 3-ethoxy4-hydroxy-benzoic acid (9), p-hydroxybenzaldehyde (10), gallic acid (11), protocatechoic acid (12), stearic acid (13), palmitic acid (14), 2, 3-dihydroxypropyl hexadecanoate (15), linoleic acid (16), scopoletin (17), indole-3-carboxaldehyde (18), 2-furoic acid (19), 5-(hydroxymethyl)-2-furaldehyde (20), (22E, 24R)-5alpha, 8alpha-epidioxy-ergosta-6, 22-dien-3beta-ol (21), (22E, 24R)-5alpha, 8alpha-epidioxy-ergosta-6, 9, 22-trien-3beta-ol (22), (+)-lariciresinol 9'-stearate (23), formononetin (24) and uridine (25). Compounds 1a, 2a, 4a and 4b were new artifacts from the separation process, and others were obtained from A. julibrissin for the first time.
Albizzia ; chemistry ; Drugs, Chinese Herbal ; chemistry ; isolation & purification ; Flowers ; chemistry ; cytology ; Molecular Structure ; Spectrometry, Mass, Electrospray Ionization

OBJECTIVETo construct a replication-defective adenovirus containing TK gene and investigate the killing effects of TK gene against human liver cancer cells SMMC-7721.

METHODSThe recombinant adenovirus ADV-TK was constructed using homologous recombination in the cells. SMMC-7721 cells transfected with recombined adenovirus were exposed to GCV, and the cell viability was measured by MTT assays.

RESULTSThe recombinant adenovirus containing TK gene was successfully constructed. Transfection by the recombinant adenovirus ADV-TK and GCV exposure significantly suppressed the growth of SMMC-7721 cells.

CONCLUSIONA replication-defective adenovirus containing TK gene has been successfully constructed, and in combination with GCV, the recombinant adenovirus produces significant killing effect against SMMC-7721 cells in vitro.

Adenoviridae ; genetics ; Cell Line, Tumor ; Genetic Therapy ; Genetic Vectors ; Humans ; Liver Neoplasms ; therapy ; Thymidine Kinase ; genetics

Objective To evaluate the in-hospital outcome of patients with calcified chronic total occlusion (CTO) lesion underwent percutaneous coronary intervention (PCI) . Methods The clinic and lesion characteristics as well as acute PCI outcome were analyzed in 726 patients with calcified CTO [624 detected by coronary angiography (CAG) and 102 detected by intravascular unltrasound (IVUS)] and received PCI therapy from June 1995 to February 2007 in our department. Results There were 728 diseased vessels with 732 lesions in these patients. Total procedure success rate (80. 6% vs. 89. 2%, P <0. 05) and the lesion success rate (80. 2% vs. 88.2%, P < 0. 05 ) were significantly lower in calcified CTO detected by CAG compared to that detected by IVUS. The causes of procedure failures in CAG detected patients were as follows: 87 guide failure, 21 balloon failure, 8 procedure related complications and 5 low TIMI blood flow (grade 2) at the end of PCI procedure. The causes of procedure failures in IVUS detected patients were as follows: 7 guide wire failure, 2 balloon failure, 1 procedure related complications and 1 patient with low TIMI blood flow (grade 2). The in-hospital major adverse cardiac events (MACE) rate was 1.1% in CAG detected calcified CTO and 1.0% in IVUS detected calcified CTO (P 0. 05). Conclusion PCI therapy resulted in satisfactory procedure success rate and in-hospital outcome for patients with calcified CTO and IVUS is helpful for further increasing the PCI procedure success rate in patients with calcified CTO.

Objective To evaluate the feasibility and efficacy of percutaneous coronary intervention (PCI) for graft occlusion post coronary artery bypass graft(CABG).Methods The clinical data of 135 post-CABG patients with bypass graft occlusion and angina peetoris symptoms admitted to our department between June 2003 and June 2007 were analyzed.The mean interval from CABG to index angiography was 33.8±23.5 months.Among 318 grafts,29 left internal mammary artery(LIMA,29/128,22.7%)and 117 saphenous vein bypass grafts(117/188,62.2%)were occluded.A total of 158 target lesions from these 146 vessels were treated with PCI.All target lesions were B2/C type lesion with 29.7%(47/158) chronic total occlusions.Results A total of 310 DES were implanted.The total success rate of PCI procedure Was 96.3%(130/135),and lesion success rate was 96.8%(153/158).No major clinical complications occurred during peri-intervention period.All Patients underwent PCI were followed at 12 month.Angiographie follow-up wag obtained in 89 patients and the angiographic restenosis rate Was 5.6% (5/89)in these patients.The major adverse cardiac events and target vessel revascularization rates were 5.4%(7/130)and 6.2%(8/130),respectively.Conclusion This study demonstrates that PCI procedure for graft occlusion post-CABG is feasible and safe and associated with a high procedure success rate and favorable long-term clinical and angiographic outcomes,