Humans ; Mental Retardation, X-Linked ; genetics ; Methyl-CpG-Binding Protein 2 ; genetics

Fast tract surgery (FTS) has been implemented in different fields of surgery to attenuate the surgical stress response and accelerate recovery.Liver resection is the preferred treatment for a variety of primary and secondary liver tumors.However,liver resection is associated with severe stress response and higher rate of postoperative morbidity and mortality.Fast tract surgery has been reported to accelerate recovery following liver resection.In this review,we summarize the recent progress of fast tract surgery in liver resection.

Objective To monitor the changes of resting energy expenditure in ventilated critically ill children,to compare the results of standard equations and indirect calorimetry (IC) in predicting energy expenditure,and to investigate the possible influence factors of the metabolic status of the critically ill children.Methods From September 2012 to September 2013,56 critically ill children on assisted ventilation and fitting the requirements of IC in pediatric intensive care unit of Shanghai Children's Medical Center were enrolled in this prospective study.IC measurements were performed using metabolic cart on day 1,4,7,10 after trachea intubation.General clinical data of these children were recorded.Results 130 IC measurements were performed in the 56 children.The measured resting energy expenditure (MREE) did not exhibit significant differences among day 1,4,7,and 10 (P =0.379).Although there were no significant differences between MREE and energy expenditure predicted with Schofield and WHO equations (P =0.917,P =0.995),the agreement was poor between the measured and predicted values (R2 =0.185,R2 =0.322).The metabolic status of the children on day 1 of ventilation was only correlated with age (P =0.000) and height (P =0.027),not with severity of underlying diseases or clinical outcomes.Conclusions MREE of IC method in ventilated critically ill children did not significantly change over time in this study.A poor agreement was observed between equationpredicted energy expenditure and MREE.IC measurement of resting energy expenditure is recommended for guiding individual nutritional support among critically ill children so as to improve clinical outcome.

Objective To analyze the nutritional status of premature neonates first fed with extensively hydrolyzed protein formula.Methods From January 2013 to December 2014, 157 premature neonates hospitalized in Neonatal Intensive Care Unit of Shanghai Children's Medical Center who were first fed with extensively hydrolyzed protein formula were enrolled.Clinical data were recorded, including related diseases, birth weight and gestational age, nutrients intake, and growth charts.Two groups were divided according to the existence or absence of feeding intolerance, and three groups were divided based on birth weight (＜ 1 500 g, 1 500 ～ 2 500 g,and ≥2 500 g).Results A total of 60 (38.2％) premature infants had feeding intolerance.The lower the birth weight and gestational age, the higher the frequency of feeding intolerance, and the incidence of feeding intolerance in ＜ 1 500 g group was 71.1％.Compared with the feeding tolerance group, the feeding intolerance group had significantly smaller birth weight [(1 620 ±440) g vs.(1 980 ±421) g, P =0.000], gestatonal age [(31.3 ±2.6) weeks vs.(33.0 ±2.1) weeks, P =0.000], birth head circumference [(28.9 ±2.2) cm vs.(30.4±1.9) cm, P=0.000], and birth length [(41.1 ±3.9) cmvs.(43.2±3.4) cm, P=0.000],but significantly longer time before transfer formula [(26.4 ± 17.6) d vs.(7.9 ± 5.3) d, P =0.000] and time before reaching sufficient feeding [(21.5 ± 10.0) d vs.(13.8 ± 6.2) d, P =0.000].The time of first feeding [＜ 1 500 g group (6.1 ±5.1) d, 1 500 ～2 500 g group (3.8 ±2.5) d, ≥2 500 g group (3.3 ± 1.2) d,P =0.002], time before transfer formula [＜ 1 500 g group (28.7 ± 18.3) d, 1 500 ～ 2 500 g group (9.7 ± 8.1) d, ≥2 500 g group (7.0 ±3.8) d, P =0.000] and time before reaching sufficient feeding [＜ 1 500 g group (24.0±10.4) d, 1 500～2 500 g group (14.3±6.0) d, ≥2 500 g group (11.4±3.5) d, P=0.000] increased along with the decrease of birth weight.The proportions of infants receiving parenteral nutrition in the feeding intolerance group (93.3％) and ＜ 1 500 g group (97.8％) were higher, with more calorie intake from parenteral nutrition [＜ 1 500 g group (325.9 ± 59.4) kJ/ (kg · d), 1 500 ～ 2 500 g group (281.2±64.8) kJ/ (kg·d), ≥2 500 g group (269.9 ±43.9) kJ/ (kg·d),P=0.001] and longer duration [＜ 1 500 g group (27.1 ± 14.5) d, 1 500 ～2 500 g group (13.0 ±7.0) d, ≥2 500 g group (8.7 ± 3.4) d, P =0.000].In terms of growth indicators, the increase in head circumference was significantly higher in the feeding intolerance group than in the feeding tolerance group [(0.7 ± 0.6) cm/week vs.(0.6 ± 0.5) cm/week, P =0.045].The increases in body weight and head circumference in the ＜ 1 500 g group were significantly higher than in the other 2 birth weight groups [body weight: ＜ 1 500 g group (21.8 ± 9.5) g/d, 1500～2500ggroup(4.2±7.6) g/d, ≥2 500 g group (4.9 ±11.9) g/d,P=0.000;head circumference : ＜ 1 500 g group (0.8 ± 0.4) cm/week, 1 500 ～ 2 500 g group (0.5 ± 0.4) cm/week, ≥ 2 500 g group (0.6 ± 0.8) cm/week, P =0.005].After controlling the variable of feeding intolerance,weight gain was negatively associated with gestational age (r =-0.666, P =0.035), birth weight (r =-0.700, P =0.024), head circumference (r =-0.846, P =0.002), and the day of returning to birth weight (r =-0.697, P =0.025), while positively associated with head circumference gain (r =0.672, P =0.033).There were no relationship between weight gain and birth length, the day of first feeding, time before transfer formula, time before reaching sufficient feeding, parenteral nutrition calorie and duration, days of hospital stay and complications.Conclusions First fed with extensively hydrolyzed protein formula, the growth in feeding intolerant premature infants may be similar to the feeding tolerant ones, which is associated with parenteral nutrition support.Premature infants with lower gestational age, birth weight, and head circumference may be more suitable for extensively hydrolyzed protein formula feeding.

AlM:To observe the efficacy of using botulinum toxin A in the treatment of blepharospasm.
METHODS: Totally 113 patients with blepharospasm were managed with a local injection of botulinum toxin A, and the therapeutic effect was evaluated.
RESULTS:Fifty-nine cases ( 52. 2%) had a complete remission of symptoms, 49 patients ( 43. 4%) presented with obvious relieved spasm, 4 cases ( 3. 5%) were partially relieved and the 1 patient ( 0. 9%) remained unchanged. The total effective rate was 99. 1%. The time of beginning effect was 1-14d. The recover time was mostly in 14d. The average of therapeutic effect lasted 1-9mo. Adverse reactions such as mild palpebra dysraphism, palpebra ptosis and local subcutaneous blood stasis were found in 23 patients, and the symptoms disappeared in 2-4wk.
CONCLUSlON:Botulinum toxin A can effectively control medium and severe blepharospasm by injecting a little dose on local muscle.

Objective To improve the understanding,the incidence and imaging findings of tuberous sclerosis complex (TSC) combined with cardiac rhabdomyomas (CRs) in fetuses and infants.Methods The imaging findings of 9 infants with TSC combined with CRs and 4 fetuses with TSC combined with CRs from our hospital between June,2006 and November,2013 were retrospectively reviewed.Results The brain MRI of 9 with TSC combined with CRs showed bilateral subependy-mal nodules,subcortical white matter and cortical tubers.Subependymal nodules were isointense or hypointense on spin-echo T1WI and hypointense or hyperintense on spin-echo T2WI.Subcortical white matter and cortical tubers were hypointense or hyperintense on T1WI and hypointense or hyperintense on T2WI.There was varying contrast enhancement.Three of 9 infants presented single cardiac tumor and 6 of 9 infants presented multiply cardiac lesions.CRs on contrast cardiac MRI showed round solid masses in ventricular septums,ventricular outflow tract,ventricle or atrial free walls.The masses were isointense relative to the cardiac muscles on T1WI,T2WI and B-TFE sequence.There was varying contrast enhancement.Four fetuses with TSC on ultrafast MRI showed bilateral multiply subependymal nodules,the nodules were isointense or hyperintense on TFE T1WI and isointense or hypointense signals on SSTSE or B-FFE sequence,Four fetuses with CRs showed isointense to hyperintense solid masses in ventricular septums on ultrafast MRI,ventricle or atrial free walls on B-FFE sequence and SSTSE sequence images.Conclusions TSC in infant and fetus is a kind of neurocutaneous syndrome,usually combines with CRs.Fetal ultrafast and routine MRI is a useful method to make a definite diagnosis for cranial and cardiac lesions.The development of MRI might improve the timeliness and accuracy of the assessment for this disease.

Objective To explore the change of dietary intake and nutritional status before and after hematopoietic stem cell transplantation (HSCT) in pediatric patients to assess the importance of nutritional interventions.Methods In this observational cohort study,65 children undergoing HSCT between January 2012 and November 2012 in the Department of Hematology and Oncology,Shanghai Children's Medical Center were enrolled.The data collected before preconditioning were considered as the baseline data.We also collected data twice a week between preconditioning and 30 days after HSCT,and once a week from 30 days to 100 days after HSCT.Dietary analysis and urea nitrogen analysis were conducted in parallel.Results The baseline level of energy intake was (5 844.9 ±2 490.4) kJ/d,protein intake was (56.4 ±28.6) g/d,fat intake was (49.7 ±38.9) g/d,and carbohydrate intake was (190.9 ± 91.1) g/d.With the hematopoietic reconstruction,the oral nutrients intake significantly decreased compared with the baseline levels (all P =0.000).During the recovery period after HSCT,the energy intake showed no significant difference when compared with the baseline level in the 6th postoperative week,protein in the 13th week,carbohydrate in the 4th week,and fat in the 6th week.The urine nitrogen was (3.9 ± 2.4) g/d before HSCT,which increased to (16.7 ± 11.0) g/d after preconditioning (P=0.000).In the 1st postoperative week,the weight (P =0.000),triceps skin fold thickness (P =0.003),mid-upper arm circumference (P =0.000),serum albumin (P =0.000) and prealbumin (P =0.000) of the patients all significantly decreased compared with the baseline levels.In the 9th postoperative week,the fat-free body weight percentage (P =0.010),muscle percentage (P =0.001) and protein percentage (P =0.000) were significantly lower than the baseline levels,while the body fat percentage was higher than the baseline level (P =0.002).Conclusions Children undergoing HSCT exhibit a marked reduction in nutrient intakes at the early stage of HSCT,which may gradually return normal during the recovery period.This process may be slow,especially for the protein,and therefore may affect the serum protein level in these pediatric patients.Thus,more careful nutrition guidance is necessary during HSCT for pediatric patients,emphasizing oral nutrients intakes,and high protein dietary or formula may be helpful.

Objective To assess the nutritional status of critically ill children and the nutritional intake during their stay in pediatric intensive care unit (PICU),and analyze the correlation factors of nutritional deftciencies.Methods One hundred and twenty-seven patients who met the PICU admission standards with a PICU stay of ＞72 h were recruited from June to October 2012 in Shanghai Children's Medical Center.Anthropometric measurements and all nutrition-related indicators of those patients were recorded from day 1 to day 10.Results 59 of the 127 patients (46.5％) showed malnutrition at admission,in which 49.2％ had severe malnutrition.65 of the 127 patients (51.2％) showed malnutrition at discharge,in which 63.1％ were severely malnourished.Median estimated energy requirements (EER) by American Society for Parenteral and Enteral Nutrition was 376.7 kJ/ (kg · d) [interquartile range,IQR:314.0～376.7 kJ/ (kg · d)],prescribed calories were 237.8 kJ/ (kg· d) [IQR:159.5 ～291.8 kJ/ (ks· d)],and delivered calories were 220.2 kJ/ (kg· d)[IQR:132.3 ～ 279.2 k J/ (kg · d)],showing significant difference (P =0.000).The delivered energy was ＜90％ of EER in 80.7％ of the 1021 recorded days and the prescribed energy was ＜90％ of EER in 74.3％of the 1021 recorded days.The cumulative calory deficiency from day 1 to day 10 in PICU was (933.5 ±745.5) kJ/ (kg · person),and the cumulative protein deficiency was (4.0 ±5.0) g/ (kg · person).83 patients (65.4％) experienced at least one feeding interruption.Altogether 170 times of feeding interruption were recorded,of which 117 (68.8％) could be explained by examination procedures.Conclusions There is a high prevalence of malnutrition in critically ill children at admission into PICU,and their nutritional status deteriorates during hospital stay.Discrepancies between required and delivered energy were mainly attributed to under-prescription,while discrepancies between prescribed and delivered energy were mainly attributed to feeding interruptions.Appropriate care for these children entails early nutritional risk screening and correct nutrition support to avoid nutritional deficiencies.

Objective To evaluate the application value of bedside lung ultrasound in diagnosis of neonatal severe pneumonia.Methods Bedside lung ultrasound and chest X-ray were performed in 59 the newborn patients admitted to the newborn department for suspected severe pneumonia,from October 2015 to March 2016.According to the characteristics of the chest X-ray,patients were divided into consolidation group (widely spot shadow in double lung and airbronchograms) and the unconsolidation group (enlargement,fuzzy texture in double lung or small patch sample changes) Differences in ultrasonic characteristics were compared between the two groups.Results Ultrasonic image characteristics were pulmonary consolidation with air bronchograms,A-line disappearance,pleural line abnormalities.Among 23 patients with chest X-ray,21 patients were found pulmonary consolidation by lung ultrasound,and all 36 patients in unconsolidation group were not found pulmonary consolidation by lung ultrasound,and there was no significant difference in the detection rate by lung ultrasound between the two groups (P＞0.05) The pulmonary ultrasound results of 20 cases in the consolidation group showed A-line disappearance,37 cases in the unconsolidation group showed A-line disappearance.There was no statistical significant differences between the two groups (P＞0.05).The pulmonary ultrasound results showed 11 cases in the consolidation group and 15 cases in the unconsolidation group with pleural line abnormalities,there was no statistically significant differences between the two groups (P＞O.05).Conclusions Ultrasound image characteristics of neonatal severe pneumonia include pulmonary consolidation and air bronchograms,A-line disappearance,pleural line abnormalities.Compared lung ultrasound and chest X-ray result,there was no difference between consolidation group and unconsolidation group.Both of the two methods can be used for evaluating the diagnosis of pneumonia.

Objective:It is demostrated that the porous protein-mineral mechanics model could provide more accurate prediction for biomaterial properties of dentine compared with the other established models.This paper would use the model to reevaluate the mechanical properties and its interacting mechanism of human dentine.Method:By using a porous proteinmineral mechanics model,the effect from the interactions between tubules,pedtubular and intertubular matrix on dentine microstructure was discussed.Results:The dentinal micromechanical properties were dependent on the tubular direction,and the absolute values of the stresses derived from the hydraulic and gas tubular pressures increased parabolically with theincreasing diameter of the tubules.It was also found that the effective elastic constants of the dentine microstructure would vary with the aging and the distribution of mineral and collagen within peritubular and intertubular matrix of detine.Conclusions:The theoretical analyses provided in this paper demonstrated that the microstructural characteristics of tubules,peritubular and intertubular dentinal matdx could have different influences on the micromechanical properties of human dentine,which showed the validity of porous protein-mineral mechanics model,and the limitation of some models that neglected the interacting mechanism.