Objective To explore the immune characteristic of a patient with hypogammaglobulinaemia and low natural killer(NK)cell numbers. Methods Case histories were analyzed repeatedly to grasp important characteristics of the disease. A routine blood examination and peripheral blood immunological detection was done. Complete neutrophil function test,lymphocyte proliferation ability and cytokine profile were evaluated. Results A 3-year-old boy was referred to the hospital because of fever and abdominal pain as well as partial intestinal obstruction. Laboratory examination revealed low immunoglobulin,reduced absolute numbers of B and NK cells and deficient B cell function of lower IFN-γ and IL-12 production. The patient also had mesenteric lymphadenopathy and a caecal mass with histology suggestive of ileocecal junctional tuberculosis. The patient was infected with cytomegalovirus(CMV)and fungus,which are not common in simple antibody deficiencies. The patient was followed for four years and during this period the patient had recurrent periodontitis resulting in the loss of one tooth. Conclusions Although the precise mechanism of the defect is unclear,we speculate that the case may cast new light on primary immunodeficiencies predominantly with antibody deficiency.(J Clin Pediatr,2011,29(7):601-608)

Objective To investigate the causes and treatment of gas?related complications during peroral endoscopic myotomy( POEM) for achalasia( AC) . Methods Retrospective analyses were performed on the clinical data of 390 AC patients receiving POEM at Chinese PLA General Hospital from December 2010 to July 2015.Univariate and multivariate analyses were conducted for potential risk factors and treatment for gas?related complications. Results Gas?related complications occurred in 55( 14?10%,55/390) cases. There were 4(1?03%,4/390)cases of pneumothorax, 29(7?44%,29/390)cases of pneumoperitoneum, 23 (5?90%,23/390)subcutaneous emphysema and 7(1?79%,7/390) cardia avulsion during POEM. All gas?related complication were cured by conservative therapy, deflation by puncture or closed drainage of chest cavity.Univariate analysis revealed that tunnel length(χ2=6?209,P=0?045),myotomy depth(χ2=12?354, P=0?002),operation time(χ2=54?905,P=0?000) and previous therapies(χ2=16?920,P=0?000) were risk factors for gas?related complications. Multivariate analysis indicated that previous therapies( OR=4?150, 95%CI:1?875?9?193 ) , tunnel length ( OR=2?410, 95%CI:1?148?5?063 ) , operation time ( OR=4?750, 95%CI: 2?196?10?283 ) were risk factors for gas?related complications for AC during POEM. Conclusion Gas?related complications occurred frequently during POEM, especially for patients with previ? ous therapies of AC. Tunnel length and operative time should be shortened as much as possible. Protective ventilation and CO2 pump should be employed during the whole POEM procedure. Mild complications, pres?ented with merely a small amount of subcutaneous emphysema, do not require any special intervention. If se?vere pneumoperitoneum and pneumothorax occur, puncture should be applied immediately.

BACKGROUND:YOUMET cervical dilating rod is made of absorbent polymer materials and has non-toxic side effects, which can avoid cross-infection in one-time use. OBJECTIVE:To observe the clinical effects of YOUMET cervical dilating rod used for cervical orifice dilation before intrauterine device insertion and removal as wel as before artificial abortion operations. METHODS:Totaly 275 female subjects schedule for cervical dilation during intrauterine device insertion and removal operations, and suction abortion for pregnancy within 10 weeks were randomly divided into two groups: 137 were included in observation group in which YOUMET cervical dilating rods were applied and 138 were included in control group in which Gongshuning glue sticks were used. Their cervical softening and dilatation situation, analgesic effect, and combined reactions during operation were observed. RESULTS AND CONCLUSION:Between the two groups, no statistical significance in general biological characteristics was found; Dilating effects in intrauterine device removing operations during child-bearing period and menopause were better in the observation group than the control group (P < 0.05). Rates of pain during insertion were higher in the observation group than the control group (P < 0.05). Rates of pain during indweling period for both groups were comparatively low, which showed no statistical significance. There was no record related to the application of cervical orifice dilating products in postoperative folow-up visit. Both products were safe with no cervical injury, slow heart rate and drop in blood pressure. YOUMET cervical dilating rod has trustworthy and safe dilating effects, which can remarkably aleviate pain.

Objective:To prepare Dermatophagoides farinae (Der f) crude protein to establish BALB/c bronchial asthma model , and to observe the morphology and degranulation of mast cells and detect related cytokines .Methods: Dermatophagoides farinae ( Der f) crude protein were prepared by trituration .30 BALB/c mice were randomly divided into 3 groups:PBS control group (A), asthma model group (B) and Der f crude protein treatment group (C).Group A were treated with PBS(100 μl) all the time, group B and group C were treated with 50 μg Der f crude protein mixed with 50μl alum adjuvant on day 0,day 7 and day 14.On day 28 group A and B were subcutaneous injected with PBS (100 μl) and group C were subcutaneous injected with Der f crude protein (350μg) in PBS(100 μl) at 1-day intervals.One week after the last treatment ,group A,B and C were intranasally challenged with 50 μg Der f crude protein daily for seven days .Twenty-four hours after the last challenge , airway hyper-responsiveness ( AHR) was assessed by using whole-body plethysmography .Two days post challenged , mice were sacrificed and bronchoalveolar lavage fluid ( BALF) was collected.Number of the total cells and eosinophil was determined .Level of IL-4,IL-10 and IFN-γcytokines in the BALF and the su-pernatant of splenocyte culture was assayed by ELISA .Level of Der f specific IgE and histamine in the sera was determined by ELISA . Airway inflammation was analyzed by HE staining .Observation of the morphology and degranulation of mast cells was analyzed by tolui -dine blue staining.Results:Compared with group B,AHR and the lung inflammation in group C were greatly reduced (P<0.01). Numbers of total cells and eosinophils in BALF of group C were significantly lower than that of group B ( P<0.01 ) .Compared with group B, the observation of degranulation of mast cells was insignificant in group C .Compared with group B(IgE:1.905), the level of specific IgE was significantly lower in groups C (IgE:1.278)(P<0.01).The level of IL-4 in BALF of group C was significantly lower than that of group B(P<0.01).Compared with group A and B, the level of IL-10 in BALF was significantly higher in group C (P<0.01) and the level of IFN-γin BALF of group C was significantly higher than that of group A and B (P<0.01).Compared with group B, the level of IL-4 in cultured splenocytes was significantly lower in group C (P<0.01), and the level of IL-10 and IFN-γin cultured splenocytes of group C was significantly higher than that of group B (P<0.01).Compared with group B, the level of histamine in BALF was slightly lower in groups C (P<0.05), and the level of histamine in sera was significantly lower in groups C (P<0.05). Conclusion:The degranulation of mast cells of murine bronchial asthma model was suppressed after the desensitization therapy .

Objective To investigate the prevalence of nutritional risk,undemutrition,overweight/obesi-ty,and apphcation of nutrition support in departments of neurology in 3 metropolitan hospitals in Beijing. Methods Adult patients in the departments of neurology from 3 metropolitan hospitals in Beijing were consecutively en-rolled from March 2005 to March 2006.Nutritional Risk Screening 2002(NRS2002)score≥3 was defined a8 nu-tritian risk,while body mass index(BMI)<18.5 ks/m2 as undemutrition.NRS2002 was performed on the next morning of admission and nutritional support evaluation was performed on the 14th day of admission or discharge day.The relationship between nutrition risk and nutrition support was analyzed.Results A total of 753 patients were enrolled.NRS2002 scoring results were obtained in 461 patients.The prevalence of undemutrition was 4.2%and nutritional risk Was 21.2%.Among them 292 cases in undemutrition category were estimated by semm albu-min<30g/L without accurate BML If the cases without accurate BMl were excluded,the prevalence of undemutri-tion was 5.4%and nutritional risk Was 10.8%.Twenty-three patients(14.4%)with NRS2002≥3 received nutrition support.Thirteen patients(2.2%)with NRS2002<3 received nutrition support.The average PN:EN ratio Wag 1:2.Conclusions A large proportion of inpatients were at nutritional risk or undemutrition in the de-partments of neurology in metropolitan hospitals in Beijing.The application of nutrition support Wag somehow inap-propriate in these patients.Evidence-based guidelines are required to improve this situation

Background and purpose:Although bortezomib has become one of the major therapeutic agents against newly diagnosed or relapsed multiple myeloma (MM), there are some patients who become resistant to bor-tezomib and then relapse, emerging as a major obstacle to long-term survival of MM patients. It has been found that elevation of intracellular cyclic adenosine monophosphate (cAMP) levels could induce cell cycle arrest and apoptosis in MM cells,which has become an interesting approach to MM therapy. This study aimed to investigate possible effects of forskolin combined with bortezomib on bortezomib-resistant myeloma cells and further explore its mechanisms. Methods:The bortezomib-resistant MM cell lines H929-R and primary cells from patients who do not respond to bortezomib were used asin vitro models. The inlfuences of bortezomib and/or forskolin on MM cells were evaluated through cellular morphology, changes of cell distribution and apoptotic rate. Meanwhile, lfow cytometry analysis was used to detect mitochondrial transmembrane potential (ΔΨm) and the expression levels of apoptosis regulators in these cells before and after the treatment were detected by Western blot.Results:Bortezomib (20 nmol/L) synergized with forskolin (50nmol/L) to induce apoptosis of H929-R cells and bortezomib-resistant primary cells. In addition, borte-zomib synergized with forskolin to induce collapse of mitochondrial transmembrane and facilitate the degradation of anti-apoptosis proteins including Bcl-2 and Mcl-1.Conclusion:Bortezomib could synergize with forskolin to induce apoptosis in bortezomib-resistant MM cells.

Objective To establish and evaluate the CaV1?1?R528H gene knock?in mouse model of thyrotoxic hy?pokalemic periodic paralysis. Methods Thirty?six 8?week?old male CaV1?1?R528H gene knock?in mice and thirty?six 8?week?old wild?type male C57BL/6J mice were used in this study. Using three?factor two?level 2 × 2 × 2 factorial design ( the three factors including mutation, thyroxine and insulin, and two levels were with or without) , the mice were divided into 8 groups. The thyroxine groups were intraperitoneally injected with levothyroxine in a dose of 350 μg/kg once per day for 12 consecutive days to produce thyrotoxicosis. The insulin groups were intraperitoneally injected with short?acting insulin in a dose of 0?8 U/kg after the last administration of levothyroxine, and the potassium levels of different groups were meas?ured and recorded before (0 min) and after insulin injection (30 min, 60 min). Results (1) Compared with the control group, the following phenomena including irritability, dull coat, increased diet and water intake, and slow body weight gain, were observed in the thyrotoxic mice. Thyroid function tests showed that the levels of T3 and T4 in the thyrotoxic mice were significantly higher than those in the corresponding control mice (P<0?05), and the TSH level was significantly low?er than that of the corresponding control mice (P<0?05 ). (2) After administration of insulin or thyroxine alone, the po?tassium levels in the mutant and wild?type mice were not significantly different. However, after combined administration of thyroxine and insulin, the potassium levels in the mutant group were significantly lower than those in the wild?type mice at 30 min and 60 min ( P<0?05 for both). (3) The main effects and interactions:Mutation factor or thyroxine factor alone did not influence on the potassium level, only insulin showed hypokalemic effect (P<0?05). There were interactions be?tween thyroxine and mutation, and between insulin and mutation (P<0?05), but no interaction between thyroxine and in?sulin. Conclusions (1) A thyrotoxicosis state in mice is successfully developed in this study. (2) An CaV1?1?R528H gene knock?in mouse model of thyrotoxic hypokalemic periodic paralysis is successfully established.

Objective To explore the cognitive status of amyotrophic lateral sclerosis (ALS) patients, and to explore the involved cognitive domains, subtypes and risk factors of mild cognitive impairment in ALS ( ALS-MCI).Methods Twenty-nine cases of ALS and 58 healthy volunteers were included.The severity of the bulbar and spinal functions of the patients was evaluated by the Improved Norris Scale.According to the Diagnostic and Statistical Manual of Mental Disorders 4th Edition-Revised( DSM-Ⅳ-R) criteria of dementia, ALS cases were classified as demented and non-demented.For non-demented ALS cases, the common cognitive batteries evaluating mental state, verbal memory, executive, attentional and visuospatial abilities were performed.Hamilton Anxiety Scale ( HAMA) and Hamilton Depression Scale (HAMD) were evaluated too.They were further classified into ALS-cognitively normal (ALS-CogNL) and ALS-MCI groups according to Petersen criteria of MCI.Risk factors possibly correlated with ALS-MCI were analyzed by comparing the differences in age, age of onset, duration of the disease, sites of onset, symptoms of bulbar and limb function between ALS-CogNL and ALS-MCI groups.Results Among 29 ALS cases, 14 (48.3% ) cases with cognitively normal( ALS-CogNL), 15 cases (51.7% ) with ALS-MCI,and none with dementia were identified.Among 15 ALS-MCI cases, 12 cases with executive dysfunction, 8 cases with memory deficits,9 cases with attention impairment and none with visuospatial impairment were found.ALSMCI cases could be further classified into three subtypes; 1 case with amnestic MCI (aMCI) ,6 cases with single domain non-memory MCI ( sdMCI), and 8 cases with multiple domains slightly impaired MCI (mdMCI).Between ALS-MCI and ALS-CogNL groups, there were significant differences (t = -2.435,- 2.576, both P ＜ 0.05) in education ((8.7 ± 2.8) years vs (11.3 ± 3.0) years) and Improved Norrisscale (bulbar score: (28.4 ± 7.7) scores vs ( 34.0 ± 3.4) scores) , however, no significant differences in sex, age, age of onset, duration,site of onset,HAMA or HAMD scores,and Improved Norris scale( spinal score) were found.Conclusions Cognitive deficits commonly exist in ALS patients.For the involved domains, executive dysfunction is the most common, deficits of attention and memory are also common, and deficit in visuospatial function is not found.The most common subtype of ALS-MCI is mdMCI.Severe bulbar symptoms and lower education may be the risk factors of ALS-MCI.

Objective To investigate the value of two-dimensional speckle tracking imaging (STI) in assessing regional myocardial dysfunction and myocardial fibrosis in a mouse model of myocardial infarction.Methods Twenty C57/B6 mice were randomly divided into two groups:myocardial infarction (MI) group (n =10) and sham-operation (SO) group (n =10).Echocardiography was performed four weeks after surgery.High frame rate two dimensional images were recorded in the left ventricular short axis views at the papillary muscle level and analysised at EchoPac workstation.Peak radical strain (PRS) and peak radical strain rate (PRSR) of each segment were measured at systolic period.Percent segmental fibrosis (PSF) was assessed from histological left ventricular cross sections stained by Masson trichrome.Results Compared with SO group,PRS and PRSR decreased significantly in all segments of MI group (P ＜ 0.01),especially in anterosepetal,anterior and lateral segments (P ＜0.05).PSF of anterosepetal,anterior,lateral and posterior segments in MI group increased significantly than those in SO group(P ＜0.01),and were negatively correlated with PRS and PRSR of these segments(r =-0.88,P ＜0.001 ; r =-0.77,P ＜0.001).Conclusions STI could accurately quantify regional myocardial function in a mouse model of myocardial infarction.Segmental radial strain and strain rate measured by STI were significantly correlated with PSF,which can be a non-invasive tool for monitoring myocardial fibrosis after myocardial infarction.

Objective To investigate the role of heme oxygenase-1(HO-1)in the protective actions of curcumin against ethanol-induced oxidative damage.Methods Microsomal HO-1 enzyme activities were determined in rat primary hepatocytes pretreated by curcumin.AST,LDH release and hepatic oxidative/antioxidant status were measured with or without ZnPPⅨ/Hemin as a classic HO-1 inhibitor/inducer,respectively.Results Curcumin could induce the HO-1 expression and enzyme activity,which was correlated with antioxidant levels in hepatocytes.HO-1 induction reached a peak under administration of 15 μmol/L curcumin for 1 h.Conclusion HO-1 induction by curcumin is contributed to the heptoprotective effects against ethanol-induced oxidative damage.