Methylmalonic acidemia(MMA) is one of the most common diseases of autosomal recessive inherited organic acid metabolic disorder,which can cause multiple organ involvement.It can be found in infants or even in the neonatal period and be manifested as feeding difficulty,repeated vomiting,convulsions,abnormal muscle tension,mental retardation,hemolytic uremic syndrome(HUS),etc.In which HUS caused by MMA is better and better known by pediatricians when treating renal diseases while the diagnosis rate is increasing year by year.This article reviews the epidemiology,etiology,pathogenesis,clinical manifestations and treatment of HUS associated with MMA.

0.05). There was significant difference between G_1, G_2and G_3(P0.05). Low expression levels in G_4 (Ⅲ,Ⅳ) were observed.Significant differences were noted between expression levels in G_4 (Ⅲ,Ⅳ) and in G_2, G_3 or G_4 (Ⅰ,Ⅱ; P

ObjectiveTo explore the effects of optional cesarean section on the level of plasma motilin and serum gastrin in umbilical blood and their relationship with gastroenteric symptoms of neonates. MethodsWe measured the concentrations of plasma motilin and serum gastrin in umbilical blood from neonates undergoing optional cesarean operation, emergency cesarean section and vaginal delivery by radioimmunoassay(RIA). ResultsFull-term deliveries undergoing optional cesarean section were with significant gastrointestinal symptoms. Plasma motilin and serum gastrin were significantly lower in neonates undergoing cesarean operation without birth initiate group(optional cesarean section)than that in cesarean section with birth initiate group and vaginal delivery group (P<0.05). ConclusionCesarean operation without birth initiate influences neonatal gastroenteric symptoms.Breast feeding which begins as early as possible will help to relieve those symptoms.

Objective:To identify the function of extracellular soluble vimentin that promotes proliferation, activation and chemotaxis of inflammatory cells. Methods: The proliferation of rat splenocytes stimulated with vimentin was evaluated in vitro. The lymphocyte counts and vimentin-antibody levels of peripheral blood in mice immunized with vimentin were detected in vivo. Peritoneal macrophages were collected and cultured with different concentrations of vimentin to detect the effect on phagocytosing chicken red blood cells. The chemotaxis of NIH3T3 fibroblast towards vimentin was observed in transwell chamber. Results:In vitro vimentin dose dependently promoted the proliferation of splenocytes. The proliferation indexes of primed and naive splenocytes cultured with 16μg/ml vimentin were reached up to ( 196. 0 ± 9. 7 )% and ( 208. 9 ± 4. 6 )% respectively without significant difference. In vivo vimentin significantly enhanced the lymphocytes number(109 L-1)of peripheral blood(5. 74±0. 51 vs. 1. 69±0. 13)and the levels of vimentin specific antibody( OD value 2. 31 ± 0. 06 vs. 0. 19 ± 0. 08 ) that shown no significant difference from immunization with vimentin plus CFA. In vitro vimentin dose dependently stimulated phagocytic ability of macrophages and performed the chemotactic effects on NIH3T3 fibroblasts. Conclusion:Extracellular soluble vimentin promotes the proliferation,activation and chemotaxis of concerned inflammatory cells and possesses the functions as an alarmin.

100 U/ml and treated for 48 h,the inhibitory ratio of the HepG2 cells was raised to (39.08?2.32)%,which were not further elevated with the increase of dose or elapse of treatment time. HepG2 cells apoptosis was induced by thrombin.in a dose-and time-dependent manner when the dose was between 25 to 100 U/ml. However,there was no significant difference in the apoptosis of different groups when the dose was higher than 100 U/ml and the time exceed 48 h. Conclusion Higher doses of thrombin can inhibit the proliferation of HepG2 cells,and lead to cell apoptosis. The both effects are increased in a dose-and time-dependent manner when the dose is

Objective To investigate the effect of different doses of ketamine on inflammatory cytokines in rabbits with severe burn at early stage and preliminarily approach its regulatory action on early stage of inflammatory reaction due to stress of trauma.Methods Forty healthy male New Zealand rabbits were randomly divided into four groups in accord with the random number table method: normal control group, scald model group, ketamine analgesia group and ketamine anesthesia group. Before scald, pentobarbital sodium was used for anesthesia, afterwards catheters were inserted into internal jugular vein and internal carotid artery respectively ready for use, and 24 hours later, Ⅲ degree scald at the animal back and buttocks occupying 30% total body surface area (TBSA) was performed as the scald model for all the rabbits except those in normal control group. In ketamine analgesia group, after scald for 0.5 hour, 0.5 mg/kg ketamine intravenous injection was given to the rabbits as the loading dosage and then persistent intravenous pump infusion of 9μg·kg-1·min-1 ketamine was applied for all together 24 hours. In ketamine anesthesia group, after scald for 0.5 hour, 1.5 mg/kg ketamine intravenous injection was given to the rabbits, and then persistent intravenous pump infusion of 45μg·kg-1·min-1 ketamine was applied for 4 hours to maintain systemic anesthesia. In normal control and scald model groups, only intravenous infusion of equal amount of normal saline was given to the rabbits. The amount of intravenous transfusion in each group and the total dosages of ketamine used in ketamine analgesia group and ketamine anesthesia group were recorded. Before scald and 0.5, 6, 12, 24 hours after scald, arterial blood gas analyses were made, and the levels of serum interleukins (IL-1, IL-6) and tumor necrosis factor-α (TNF-α) were determined.Results Although the indexes of blood gas analysis were changed in the four groups, they were all in the normal range, showing that the respiratory function was in the normal range and indirectly reflecting that the circulatory function was also in the normal range, thus the effects on cytokines by factors of respiratory and circulatory functions were ruled out. The levels of IL-1, IL-6 and TNF-α before scald showed no statistically significant differencesamong the four groups (allP > 0.05). From 0.5 hour after scald, the levels of IL-1, IL-6 and TNF-α were markedly higher in model group than those of normal control group [IL-1 (ng/L): 30.27±0.93 vs. 13.79±1.11, IL-6 (ng/L): 47.22±1.49 vs. 46.31±4.12, TNF-α (ng/L): 243.39±20.85 vs. 190.95±14.97, allP < 0.05], and the situation continued until 24 hours after scald; the levels of IL-1, IL-6 and TNF-α from 6 hours after scald were significantly decreased in ketamine analgesia and ketamine anesthesia groups compared with those in the model group, and from 12 hours after scald, the degrees of descent in levels of the above indexes in ketamine analgesia group were more obvious than those in ketamine anesthesia group [IL-1 (ng/L): 19.28±2.51 vs. 40.12±10.31, IL-6 (ng/L): 52.10±4.23 vs. 72.20±10.11, TNF-α (ng/L): 246.03±20.74 vs. 313.71±27.34, allP < 0.05].Conclusion The low-dose ketamine analgesia and ketamine anesthesia have certain degree of inhibitory effect on the expression and release of inflammatory cytokines at the early stage in rabbits with severe burn, the effect of long-term low-dose ketamine analgesia being more significant.

This review analyzed the effect of folic acid on pemetrexed efficacy and toxicity, especially high-does folic acid supplementation. The optimal duration for supplementation prior to the first dose of pemetrexed has not been defined. The review also explored whether total plasma homocysteine levels can be as a marker to predict and avoid toxicity from pemetrexed therapy.

Objective To evaluate neurodevelopmental outcome of very preterm(gestational age ＜ 32 weeks) and very low birth weight infant (VLBWI) (weight ＜ 1 500 g) and to examine the effectiveness of an early intervention program till 12 months corrected age.Methods Seventy followed-up very preterms and VLB-WI in Jinan Maternity and Childcare Hospital were enrolled in this study from January 2012 to and were divided into two groups by birth weight.All infants received 20 items of behavioral neurological assessment at 1 to 12 months corrected age and tested mental and psychomotor development with the use of CDCC at 6,12 months corrected age.The preterms who were abnomal in the 20 items of behavioral neurological assessment would receive early intervention (including kinesitherapy, physiotherapy, cereal circulation therapeutic equipment) by physiotherapists and their parents who received an intervention program training and were strongly encouraged to participate in the intervention sessions.The intervention method was adjusted according to the neurological assessment.The SPSS statistical software package for Windows, version 15.0, was used to run Fisher's exact test and t-test on the data presented,and P value of less than 0.05 was regarded as statistically significant.Results The average gestational age of infants was (30.4 ± 1.8) weeks,and average birth weight (1 463.7 ± 307.5) g.The incidence of extrauterine growth restriction was 57.1％ at first follow-up.The incidence of neurodevelpmental impairment NDI) and cerebral palsy tendency at 6 corrected months were 14.3％ ,8.6％ respectively.At 12 months corrected age,the incidence of NDI decreased to 2.9％ and cerebral palsy to 2.9％.There was significant difference in the incidence of NDI between 6 and 12 corrected months.There was no significant difference in the incidence of psychomotor developmental index ＜ 70, mental developmental index ＜ 70, NDI and cerebral palsy between the two groups.Conclusion The early intervention program can improve VLBWI neurodevelopmental outcomes at 12 months' corrected age and reduce the incidence of cerebral palsy.

Objective To investigate the short and long term effects of Sunmax injection for the rhytidoplasty on different parts of the fa-cial wrinkles. Methods 1 028 face lift patients were collected from January 2007 to December 2011 who admitted to our plastic surgery de-partment,236 cases of male,792 cases of female,with an average age of 48. 7 (21~72)years old. According to different drug injection sites, all the patients were divided into 5 groups:wrinkles between eyebrows,the forehead wrinkles,nasolabial fold wrinkles, crow's feet,nose wrin-kles. The rhytidoplasty effects of 5 groups a day,1 month,3 months,6 months,9 months after injection were observed and compared. Further-more,the short and long term effects of the Sunmax injections were integrated to analyze. Results After Sunmax injection,a variety of skin wrinkles and sags disappeared;1 month after injection,wrinkles did not appear;3~6 months after injection,the effects was still as the same as the day after injection. 9 months after injection,the effects for the forehead wrinkles and crow's feet was slightly diminished. Conclusion Sunmax injection is an emerging technology to remove facial wrinkles,which has been widelymused inmour department for severalmyears and got patients' postoperative satisfaction. It is worth spreading for rhytidoplasty.

Objectives To explore the clinical manifestations, treatment and prognosis of a case of blindness caused by nephrotic syndrome with cerebral venous sinus thrombosis (CVST). Methods The clinical manifestations, diagnosis and treatment of a case of NS with CVST were analyzed. The latest domestic and foreign reseach progresses in treatment for CVST in children were reviewed. Results Epilepsy suddenly appeared with diplopia, binocular vision loss and blindness in anticoagulant therapy for the child with NS. Brain magnetic resonance venography (MRV) suggested CVST. MRV reexam-ined showed that the intracranial thrombosis was completely dissolved after urokinase thrombolysis for one month followed by ineffective heparin anticoagulation. At present, international standards of anticoagulant therapy have been adopted in the treatment for CVST patients. Coagulation function (e.g.APTT) and international standardization ratio were monitored in order to prevent bleeding. Conclusions It is better to perform neural imaging examination early in suspected CVST patients. Anti-coagulation and thrombolytic therapy should be given immediately once the risk of bleeding was excluded and used for 3-6 months.