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

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.

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 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.

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 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 explore the effect of aminolevulinic acid-based photodynamic therapy(ALA-PDT) on alloreactived peripheral blood mononuclear cells(PBMCs).Methods Human PBMCs from different healthy donor were collected and mixed in the one-way mixed lymphocyte culture(MLC) for 5 days. The cells were harvested and aminolevulinic acid(ALA) were added into ALA group and ALA+Light group with ultimate concentrations of 0.5 mmol/L,1.0 mmol/L,1.5 mmol/L,2.0 mmol/L and 2.5 mmol/L.After cultured for 2 hours, 4 hours and 6 hours respectively in 37 ℃ 5% carbon dioxide incubator,Light group and ALA+Light group were irradiated by light of 410 nm wavelength for 1 hour.The MLC cells were treated with the former stimulator cells for 48 hours.The survival of stimulator cells were detected using MTT colorimetric assay and the kill rates of treated cells were calculated.Results The kill rate of ALA+Light group on stimulators was apparently lower than those of Light group, ALA group and control group, (33.0?26.5)% vs (87.1?2.2)%,(89.2?2.5)%,(90.3?1.9)%(All P

Purpose To investigate the clinicopathological features of adolescent mixed epithelial and stromal tumor of the kidney ( MESTK) and improve recognization of this rare disease. Methods Clinicopathological and immunohistochemical characteristics of MESTK occured in 17-year-old girl were studied. Reviewed the related literatures, clinical and pathological characteristics of adoles-cent MESTK were analysed comprehensively. Results Congenital perineal spill was the main clinical manifestations. Microscopically, the tumor showed nodules and was composed of a mixture of epithelial and stromal elements. Glands were lined with columnar or cilia-ted columnar cells. Stromal cells surrounding glands seemed like ovaries and away from glands seemed like the differentiation of smooth muscle. Immunohistochemical staining revealed that the epithelial cells were positive for CK7 and vimentin. Stromal cells expressed desmin, smooth muscle actin ( SMA) , ER and PR. It was noteworthy that stromal cells away from glands expressed desmin. Conclu-sions As a kind of rare benign neoplasm of kidney, MESTK often occurs in perimenopausal women. But MESTK can also occur in ad-olescence, and has nothing to do with the use of hormone. Therefore, the renal tumor occurred in teenagers with biphasic differentiation should be differentiated from MESTK.

Objective To explore the effect of morroniside on blood viscosity in focal cerebral ischemia/reperfusion in rats. Methods The animal model was induced with occlusion of middle cerebral artery (MCAO), and morroniside (30 mg/kg, 90 mg/kg, 270 mg/kg) was then administered intragastrically for 7 d. Whole blood viscosity and plasma viscosity were detected with auto-hemorheological instrument and microplasma testing device. Results Compared with sham-operated group, both whole blood viscosity and plasma viscosity significantly increased in ischemic rats (P<0.001); however, morroniside reduced whole blood and plasma viscosity noticeably (P<0.001). Conclusion Morroniside can inhibit the increase of blood viscosity induced by focal cerebral ischemia/reperfusion in rats.

The pathways of brain injury caused by ischemic stroke are complicated. Due to those largely ineffective current therapeutic strategies, the development of new and efficient therapeutic interventions is clearly needed. This article summarized the progress in the pathogenesis of ischemic stroke, the drugs for treatment and the therapeutic time window.