Objective To observe the clinical characteristics of 2 patients with mucolipidosis type Ⅲ. Methods Two sibling patients (a sister and a brother) with mucolipidosis type Ⅲ symptoms and other family members were the research objects, and the changes of their features of hand bone imaging, blood indexes [blood glucose, cholesterol, triacylglycerol (TG), total protein (TP), albumin (Alb)] and body composition were analyzed. Results Except the 2 patients, the bone morphology, blood indexes and body compositions in other 7 family members were under normal conditions. The phalanx intervals of both hands in 2 patients with mucolipidosis type Ⅲwere widened significantly, among them the thumb manifestation was more obvious; the distal segments of phalanxes in both hands became pointed and curved presenting a "claw-like hand" deformity; the metacarpal and distal carpal metaphysis were obviously enlarged, and scaphoid, lunate, trianglar, orbicular, and trapezium and trapezoid bones were loosely arranged at the wrist; the distal ends of ulna and radius were markedly enlarged. Compared to healthy people, the triglyceride levels of serum in the 2 patients were obviously reduced (the percentage of reduction: 57.14% and 41.07% respectively); body mass indexes (BMI), total fat and visceral fat were significantly lowered (BMI reduction percentage:26.81% and 14.55%, total fat reduction percentage: 38.12% and 44.95%, visceral fat reduction percentage: 62.25% and 67.74%, respectively) in the two patients. Conclusion The purpose of studying the biochemistry indexes, imaging characteristics and body compositions is to more deeply understand the clinical symptoms and signs of the 2 sibling patients with mucolipidosis type Ⅲ in a family to provide a theoretical reference.

Objective To evaluate the effect of obstructive jaundice on the accuracy of left ventricular end-diastolic volume (LVEDV) and stroke volume variability (SVV) in monitoring fluid responsiveness.Methods Thirty patients of both sexes,aged 45-60 yr,weighing 55-70 kg,of American Society of Anesthesiologists physical status Ⅰ or Ⅱ,with New York Heart Association Ⅰ,scheduled for elective pancreatoduodenectomy,were divided into 2 groups according to the serum total bilirubin levels:A group (serum total bilirubin ≥ 17 μmmol/L,n =16) and B group (serum total bilirubin＜ 17 μmmol/L,n =14).Six percent hydroxyethyl starch 500 ml was infused over 40 min after anesthesia induction.The parameters of VigileoTM such as cardiac output (CO),SVV,systemic vascular resistance (SVR) and pulmonary capillary wedge pressure and indices measured by transesophageal three-dimensional echocardiography such as LVEDV,left ventricular end-systolic volume,CO',left ventricular ejection fraction (LVEF) and ratio of mitral peak velocity of early filling (E) to early diastolic mitral annular velocity (e',E/e'ratio) were recorded before and after fluid loading.Results Compared with that before fluid loading,SVV was significantly decreased in two groups,and CO,LVEDV,CO'and LVEF were significantly increased in group B,and E/e'ratio was significantly increased in group A (P＜0.05).Compared with group B,CO,SVR,CO'and LVEF were significantly decreased,and pulmonary capillary wedge pressure was increased in group A (P＜0.05).Conclusion Obstructive jaundice causes decrease in the accuracy of LVEDV in monitoring fluid responsiveness and no effect on SVV.

Objective To investigate the differences of hand and foot morphology and genetic phenotypic characteristics in adults of Hui and Han nationality in Southwestern Henan.Methods The indicators of height,weight,hand and foot were measured by the morphological measurements,the hand and foot genetic phenotype classification was observed and performed the statistical analysis.Results The hand width,foot length and foot width of the Hui adult men and women in Southwestern Henan were(8.27±0.55,23.10±1.20,9.34±0.83)cm and(7.41±0.44,20.50±1.23,8.79±0.69) cm,respectively,while which of the Han adult men and women were(8.56±0.09,24.57±1.33,9.47±0.70)cm and(7.74±0.36,22.46±1.21,8.91±0.85) cm,respectively.The total number of both hands fingerprint ridge line in Hui adult men and women were(135.06 ± 19.87) and (125.50 ±20.44)respectively,and which in Han adult men and women were (144.46 ±14.08) and (129.20 ± 20.34)respectively,the difference was statistically significant(P＜0.05).The tPD,atd angle and a-b crest line number among the Hui and Han nationalities were 16.07± 6.46,(44.61±8.66)°,34.04±5.47 and 16.53±6.27,(43.19±9.52)°,36.73±4.22 respectively.And the handedness,fingernail form,thumb type,footedness,right type ratio of foot and toe length of the Hui and Han nationalities were 90.01％,38.52％,85.59％,70.47％,56.92％ and 89.33％,45.26％,70.91％,96.98％,74.89％,respectively,the difference between the Hui and Han nationalities was statistically significant(P＜0.05).Conclusion The national differences and gender differences exist in the multiple indicators of hand and foot morphology,finger and palm prints,and genetic phenotype among the Hui and Han adults in Southwestern Henan.

Objective To investigate the effects of different doses of dexmedetomidine on perioperative inflammatory responses in patients undergoing one-lung ventilation (OLV).Methods Thirty-six ASA T or Ⅱ patients (aged 43-72 years and weighing 50-78 kg) scheduled for esophagectomy were randomly divided into three groups (n =12 each):control group (group C),low dose dexmedetomidine group (group D1) and high dose dexmedetomidine group (group D2).Dexmedetomidine 1 μg/kg was infused intravenously 10 minutes before anesthesia induction,then infused at a rate of 0.2 μg· kg-1 · h-1 (group D1) or 0.5 μg· kg-1· h-1 (group D2) until 30 minutes before the end of operation.Group C received the equal volume of normal saline.Blood samples were collected before anesthesia induction (T0),immediately before OLV (T1),30 minutes after OLV (T2),90 minutes after OLV (T3),30 minutes after lung inflation (T4) and 2 hours after operation (T5) for monitoring serumlevels of tumor necrosis factor-alpha (TNF-α) and interleukin-8 (IL-8).Results Compared with T0,serum levels of TNF-α and IL-8 significantly increased at T3 and T5 in all the three groups (P ＜ 0.05).Compared with group C,serum levels of TNF-α and IL-8 significantly decreased at T3 and T5 in group D2 (P ＜ 0.05).There was no significant difference in the indexes mentioned above between group C and group D1 (P ＞ 0.05).Conclusion Dexmedetomidine 1 μg/kg given before anesthesia induction and then infused at the rate of 0.5 μg· kg-1 ·h-1 during operation can reduce inflammatory responses in patients undergoing OLV.

Objective:To investigation of the long-term-siRNA treatment with HBV transgene mice on inhibit replication of hepatitis B virus .Methods:The constructed siRNA expressed vectors was transfected HBV transgene mice by hydrodynamics -based in-jection via vena caudalis .Different groups were set including:specificity siRNA groups ( pSilencer5.1/C2,pSilencer4.1/C2,pSilenc-er3.1/C2),PBS group and negative vector group (n=10).The effect was observed in different periods (6 d,21 d,1 months,3 months, 6 months and 9 months after injection ) .HBsAg was analyzed by Chemiluminescence method , HBV-DNA was analyzed by real time quantitative PCR ( RQ-PCR) .Results:Compared with the PBS group , specificity siRNA groups showed decreased levels of HBsAg and HBV-DNA (P<0.05).Negative vector group did not show such changes ,there were no significant differences (P>0.05).Conclu-sion:The siRNA based on the expression vector can suppress the expression and replication of HBV in HBV transgene mice .The inhi-bition effects of long-term-siRNA treatment was specific .

Objective To investigate the effect of doxapram on inhibition of medullary respiratory center excitability by sevoflurane in rats.Methods Neonatal Sprague-Dawley rats of both sexes,aged 1-4 days,were used in this study.Isolated medulla oblongata-spinal cord specimens were made according to the method described by Suzue and perfused with the artificial cerebrospinal fluid saturated with 95％O2-5％CO2.The specimens were randomly divided into 3 groups ( n =9 each):control group (group C),sevoflurane group (group S) and sevoflurane + doxapram group (group S + D).Respiratory rhythmical discharge activity of the hypoglossal nerve rootlets was recorded using suction electrode.After 10 min of equilibration,the specimens were perfused with the artificial cerebrospinal fluid,5％ sevoflurane and the mixture of 5％ sevoflurane and 5 μmol/L doxapram for 10 min in groups C,S,and S + D respectively.The respiratory cycle,inspiratory time and integral amplitude of inspiratory discharge were recorded.Results Compared with group C,the respiratory cycle was significantly prolonged,the inspiratory time was significantly shortened,and the integral amplitude of inspiratory discharge was significantly decreased in group S (P ＜ 0.05),and no significant change was found in the parameters mentioned above in group S + D (P ＞ 0.05).Compared with group S,the respiratory cycle was significantly shortened,the inspiratory time was significantly prolonged,the integral amplitude of inspiratory discharge was significantly increased in group S + D ( P ＜ 0.05).Conclusion Doxapram antagonizes sevoflurane-induced inhibition of excitability of medullary respiratory center in rats.

Objective: To explore the main characteristics of syndromes in traditional Chinese medicine (TCM) in post-stroke depression (PSD) and to provide basis for treatments with TCM herbs. Methods: According to diagnostic criteria of PSD, stroke patients and depression patients from Department of Neurology, First Affiliated Hospital, Anhui University of Traditional Chinese Medicine were assigned into cerebral stroke group (150 cases), depression group (151 cases) and PSD group (123 cases). Neuropsychological assessments and imaging and biochemical analyses were conducted. TCM syndrome differentiation for these diseases was performed. We also determined the characteristics of TCM syndromes of PSD, relative risk of the syndromes and their correlations with ages as well. Results: Scores of qi stagnation and blood stasis, liver qi depression, and transformation of fire due to qi stagnation in PSD group were significant higher than those in cerebral stroke group (P<0.05, P<0.01). In cerebral stroke group, majority of the patients displayed one syndrome, while in PSD and depression groups, the patients had three or more syndromes. Of these syndromes, the incidence rate of syndrome of liver qi depression complicated with transformation of fire due to qi stagnation or flaring of fire due to yin deficiency was high. The syndrome of liver qi depression occurred much more frequently in PSD group and depression group than in cerebral stroke group (P<0.05, P<0.01). The logistic regression analysis showed that the syndrome of qi stagnation and blood stasis had high relative risk to PSD. The syndrome of deficiency of heart and spleen was positively correlated with age in cerebral stroke group. Conclusion: The main TCM syndromes of PSD and depression are qi stagnation and blood stasis, liver qi depression, and transformation of fire due to qi stagnation. The syndrome of deficiency of heart and spleen is closely related to age among the stroke patients. The syndrome of qi stagnation and blood stasis serves as an independent risk factor for PSD. The more complicated the syndromes are, the more serious depression becomes.

Objective To study the effects of asiaticoside on the growth of melanoma B16 cell cultures in vitro. Methods Melanoma B16 cells were subcultured and the inhibition of cellular growth was investigated. The morphology of the cells was observed after inhibition. The induction of apoptosis by asiaticoside was determined by flow cytometry. Results It was found that asiaticoside could significantly inhibit the growth of B16 cell cultures in vitro in a dose-dependent manner. The annexin-v positive cells were increased, along with that cells intaking R123 marked mitochondria were decreased, and PI positive cells increased, which indicated that cellular apoptosis was induced. Conclusion Asiaticoside plays an inhibitory role in the growth of melanoma B16 cells.

Objective To report the first case of cervical l ymphadenitis caused by Candida albicans in China.Methods A series of clinical,histopatholog ic.and mycologic studies were carried out in an 8year-old-boy with cervical lymphadenitis,who had no definite underlying disease.Candida albicans was isolated fromthe patient.The lymph node was investigated by me ans of culture,pathological and tra nsmission electron microscopic examination and cellular immunology detected by flow cytometer.Results There were several enlarged,warm,a nd fluctuant cervical lymph nodes which varied fr om 1cm?1cm to 4cm?4cm in size.One swo llen node was excised,and Candida albicans was found in the culture.The histopathologic changes of the cervical lym ph node were compatible with chronic granulomatous disease.PAS and methenamine silver(PAM)stain of a touch preparation of the bi opsy specimen revealed budding spores an d pseudohyphae.Lymphopenia with a p roportionate decrease of T-helper a nd T-suppressor cells was shown with flowcytometry.The patient received a combination therapy of surgery,flu conazole,thymosin and so on.After one month,t he patient's condition was improved.Many small lymph nodes disappeared,large lymph nodes became smaller.Tw o months later,only one large lymph n ode could be touched.Conclusion It is the first case of cervical lymphad enitis caused by Candida reported in China.Combination therapy of surgery,fluconazole and thymosin is effective.

In this study, the recombinant plasmid pPFl4 labeled with photobiotin was used as a probe to detect the patients infected with Plasmodium falciparum ( P. f.) by dothybridiza-tion. The results showed that out of 35 cases with P. f., 29 were positive, 5 were negative and one was doubtful. One patient with P.f. and P. vivax mixed infection showed positive result. The total positive rate was 83. 3% (30/36). 3 out of 33 normal human blood samples were positive, so the false positive rate was 9%. In addition, there was a correlation between the positive rate of detection and parasitaemia level. The detection sensitivity was 5 ?10-5.