Objective To investigate the clinical features of eleven cases of acute liver failure as the initial presentation of hemophagocytic syndrome(HPS),in order to improve the early diagnosis.Methods Eleven cases of acute liver failure as the initial presentation of HPS admitted in PICU of Shengjing Hospital affiliated to China Medical University from September 201 1 to February 2015 were investigated,the clinical manifestations,laboratory findings,therapy methods and prognosis were analyzed.Results Eleven cases of HPS had the initial symptom of acute liver failure accompanied by severe coagulation abnormalities,increase of alanine aminotransferase and aspartate aminotransferase,decrease of fibrinogen.All 1 1 cases with speno-megaly had more than 1 week thermal process.Glucocorticoid and gamma globulin were used to inhibit the activation of monocyte-macrophage cell system.Chemotherapy such as Etoposide were used as the basic treat-ment in the early stage.Plasma exchange and continuous hemodialysis and filtration were used in severe cases with bleeding tendency.One of these 11 children survived,4 cases died of multiple organ dysfunction syn-drome,and discharged six cases were followed up for mortality.Conclusion Unexplained acute liver failure, fever and cytopenias may suggest HPS,the mortality rate can be reduced by early diagnosis and treatment.

Objective To investigate the clinical features and risk factors in children with symptomatic central venous catheter-related deep vein thrombosis,and to provide guidence for clinical therapy.Methods The clinical data of 105 children with central venous catheter were retrospectively analyzed.According to the thrombosis or not,these children were classified into two groups:thrombosis group and non-thrombosis group.The risk factors influencing symptomatic central venous catheter-related deep vein thrombosis forming were identified by Logistic regression analysis.Results Among the 105 cases with central venous catheter,the male to female ratio was 68:37;age ranged from 8.5 months to 13 years old with average age(5.5 ±4.0) years old.There were 98 cases in non-thrombosis group and 7 cases in thrombosis group.Factors such as age[(5.7 ±4.1)years old vs.(2.5 ± 1.8) years old],central venous catheter dwell time[(6.1 ±2.3)d vs.(8.9 ± 2.1) d],more than 7 days parenteral nutrition application (11/98 cases vs.5/7 cases) and more than 7 days intravenous application of mannitol(7/98 cases vs.4/7 cases)were found significantly different between the thrombosis group and non-thrombosis group(P ＜ 0.05).Multivariate Logistic regression analysis showed that more than 7 days parenteral nutrition application and intravenous mannitol were the risk factors of symptomatic central venous catheter-related deep vein thrombosis [OR =50.703 (95 ％ CI 3.258-789.056),OR =15.590 (95 ％ CI 1.196-203.146),P ＜ 0.05].Conclusion Symptomatic central venous catheter-related deep vein thrombosis is a common complication of deep venous catheterization.It cause acute pulmonary embolism and some critical diseases,and influence the prognosis and prolong hospital stay.Application of intravenous nutrition more than 7 days and intravenous mannitol more than 7 days are the risk factors of symptomatic central venous catheter-related deep vein thrombosis.

Objective To investigate the clinical manifestations ofi nfantile and late-onset glycogen sot rage diseaes type Ⅱ.Mte hods We analyzed the cliin calm anifestations and prognosiso f infantile and late-onset glcy ogen storage disease type Ⅱ with a retrospective analysis of five cases admitted in PICU of Shengjing Hospital of China Medical University from 2013 to 2014.Resulst Firsts ymptoms of three infan-tile cases were dyspnea,cardiac hypertrophy,hepatomegaly,skeletal muscle weakness and low concentration of α-glucosidase A.Two cases completed gene detection.One case had frameshift mutation and missense mu-tation,and the other had two missense mutatoi n.Three infantile csa es all showed arrhythmia performance. Two cases died of fat l arrhythmia.One caes received ne zyme replacement therapy and survived.The main symptoms of two al te-onset cases who had not get gene detection were dyspnea,low muscle strength,muscle hypotonia and low concentration of ca idα-gluco sidase.One case receivedm echanicla ventilation,complicated with multiple infections,severe pneumonia andv entilator dependence,finally gave up the treatment.The other died of cardiac arrhythmia.Concluis on Infantile cases have the major symptoms of myocardial hypert o-phy,hepatomegaly,low muscular tension with rapid progression,high mortality and fatal arrhythmia.Late-on-set cases have the clinical features of respiratory failure,proximal limb muscle weakness and be susceptible to ventilator dependence and multiple infections.Enzyme replacement therapy can improve the clinical symp-toms of infantile cases.

Objective To investigate the diagnostic value of plasma B-type natriuretic peptide(BNP) in left to right shunt congenital heart disease accompanied by heart failure in PICU.Methods We retrospectively reviewed the clinical data of 52 cases diagnosed left to right shunt congenital heart disease in the PICU of Shengjing Hospital of China Medical University from January 2012 to June 2014.The cases were divided into negative control group(n=18) and heart failure group(n=34) according to the criteria for the diagnosis of pediatric heart failure.We respectively compared plasma BNP,size of heart defects,left ventricular end-diastolic volume index(LVEDVI),ratio of left ventricular early diastolic filling blood flow velocity and left ventricular late diastolic filling blood flow velocity(E/A),left ventricular ejection fraction(LVEF),pulmonary artery systolic pressure(PASP),and cardiothoracic ratio between the two groups.We analyzed the correlation between plasma BNP and the size of heart defects,LVEDVI,E/A,cardiothoracic ratio,LVEF,PASP.The receiver operating characteristic curve was used to determine the optimal cut-off value of plasma BNP to diagnose heart failure.Results Plasma BNP were 87.7(22.7,165.7)pg/ml in negative control group and 716.5(326.8,1813.0)pg/ml in heart failure group.The plasma BNP level of heart failure group was significantly higher than that of negative control group(Z=5.3,P<0.01).Size of heart defects were 5.0(3.0,6.8) mm in negative control group and 7.4(5.5,9.0)mm in heart failure group.Size of heart defects of heart failure group was significantly higher than that of negative control group(Z=3.5,P<0.01).LVEDVI were (44.6±18.3)ml/m3 in negative control group and (70.8±38.4)ml/m3 in heart failure group.LVEDVI of heart failure group was significantly higher than that of negative control group(t=2.7,P=0.01).E/A were 1.3±0.3 in negative control group and 1.1±0.3 in heart failure group.E/A of negative control group was significantly higher than that of heart failure group(t=2.2,P=0.04).Plasma BNP had a positive relation with cardiothoracic ratio(r=0.49,P=0.01) and a negative correlation with E/A(r=-0.28,P=0.04).The optimal cut-off value of plasma BNP was 181.8 pg/ml.The sensitivity of diagnosis of heart failure was 94% and the specificity was 88%.The area under the receiver operating characteristic curve was 0.951.Conclusion Plasma BNP may comprise a sensitive marker for heart failure of left to right shunt congenital heart disease.It is recommended that 181.8 pg/ml is the optimal cut-off value to diagnose heart failure of left to right shunt congenital heart disease.

Objective: To investigate the epidemiology and clinical features of pulmonary hemorrhage in children at pediatric intensive care unit(PICU). Methods: A retrospective cohort case analysis was performed in order to investigate the incidence, primary disease, clinical manifestations and clinical characteristics of pulmonary hemorrhage in children at PICU of Shengjing Hospital of China Medical University from January 2008 to September 2017. Results: Among the 73 cases, 39 cases were male (53.4%) and 34 cases were female(46.6%), and the average age was 31(5, 72)months.The conditions of 14 cases(19.2%) were improved and discharged, 17(23.3%) cases died during the hospitalization period, and 42 cases gave up treatment halfway.The primary causes of pulmonary hemorrhage included severe heart failure in 17 patients (23.3%), acute respiratory distress syndrome (ARDS) in 12 patients (16.4%), severe sepsis in 10 patients (13.7%), hematological diseases in 10 patients (13.7%), intracranial hemorrhage occurred in 5 patients (6.8%), cardiac arrest in 5 patients (6.8%), coagulation dysfunction in 5 patients(6.8%), renal disease in 3 patients(4.2%) and other diseases in 6 patients(8.3%). The symptoms of all cases (73 cases) of pulmonary hemorrhage included dyspnea, moist rales and decreased blood oxygen saturation.Nasal bleeding or coffee ground like material drawn out from nasal stomach tube could be found in 32 cases(43.8%, 32/73 cases), 36 cases(49.3%, 36/73 cases)were complicated by shock and 9 cases (12.3%, 9/73 cases) were combined with convulsions, and 47 cases (64.4%, 47/73 cases) were given cardiopulmonary resuscitation.The positive end expiratory pressure in ARDS group was(10.2±2.4) cmH₂O(1 cmH₂O=0.098 kPa), which was higher than that in other groups (F=4.59, P<0.01). The oxygen concentration in acute respiratory distress syndrome group was(0.9±0.1)% , which was higher than that in other groups (F=3.16, P<0.01). International normalization ratio was 1.9±0.7 in severe sepsis group, which was higher than that in other groups (F=2.96, P<0.01). Blood platelets in hematological disease group was (52.0±46.8)×10⁹/L, which was lower than that in other groups (F=8.23, P<0.01) and hemoglobin in hematological disease group was (81.6±15.6) g/L, which was lower than that in other groups (F=5.48, P<0.01). Chest X-ray examinations showed decreased lung permeability, multiple exudative lesions in 61 cases and increased bronchovascular shadows in 5 cases. Conclusions Pulmonary hemorrhage may be a complication of many diseases. Severe heart failure, ARDS, severe sepsis and hematological diseases are the first four primary causes of pulmonary hemorrhage at PICU. Different causes of pulmonary hemorrhage manifest different characteristics.If the symptom of a patient shows heart rate increase, dyspnea, moist rales and blood oxygen saturation decreased with bleeding tendency and pulmonary infiltration by chest X-ray, he can be suspected of pulmonary hemorrhage.

Objective: To investigate the incidence and clinical characteristics of new-onset organ dysfunction of patients in pediatric intensive care unit (PICU). Method: A retrospective observational study identified all patients admitted to the PICU of Shengjing Hospital Affiliated to China Medical University from January 2015 to January 2016. The functional status score (FSS) was evaluated at admission and hospital discharge respectively, and the difference defined as ΔFSS between the FSS at hospital discharge and the FSS at admission was calculated. According to the initial FSS, the patients were divided into normal group (6-7 scores), mildly abnormal group (8-9 scores), moderate abnormal group (10-15 scores), severe abnormal group (16-21 scores) and extreme severe abnormal group (22-30 scores). According to the primary disease, all cases were divided into cardiovascular disease group, urinary disease group, surgery group, digestive disease group, neurological disease group, respiratory disease group, hematological disease group, poisoning group and other group. According to the FSS domain, all cases were divided into mental status group, sensory group, communication group, motor group, feeding group, respiratory group. The incidence of new-onset organ dysfunction, the case fatality rate and the FSS of each group were calculated. Comparisons were performed using a chi-square test, t test and analysis of variance. Result: The study population included 928 patients (561(60.5%) male, mean age (31.1±1.3) months). The incidence of new-onset organ dysfunction was 8.8%(82/928) and the case fatality rate was 1.3%(12/928). The FSS at hospital discharge(scores), ΔFSS (scores) and the incidence of new-onset organ dysfunction were significantly less in patients in the normal group (6.38±0.17, -0.20±0.17 and 1.3%(3/229), respectively) compared to patients in the mildly abnormal group (7.09±0.27, -1.39±0.27 and 7.2%(12/170), respectively, t=2.36, 3.93, χ²=7.39, all P<0.05), patients in the moderately abnormal group (8.86±0.28, -2.76±0.28 and 10.6%(38/359), t=6.56, 6.91, χ²=17.14, all P<0.05), patients in the severely abnormal group(13.56±0.88, -4.39±0.88 and 24.6%(19/79), t=12.29, 7.13, χ²=42.43, all P<0.05) and patients in the extreme severely abnormal group(18.68±0.99, -6.59±0.91 and 10.9%(10/91), t=18.15, 10.10, χ²=13.27, all P<0.05). Significant difference was found regarding the incidence of new-onset organ dysfunction among patients in cardiovascular disease group (27.3%, 24/88), surgery group (9.2%, 6/65), digestive disease group (8.2%, 8/97), neurological disease group (7.7%, 23/299), respiratory disease group (6.9%, 17/248), hematological disease group (3.9%, 2/51) and toxic group (0, 0/61) (χ²=37.75, all P<0.05). There were significant differences among primary disease groups regarding the FSS at admission, the FSS at hospital discharge, ΔFSS, Δmental status FSS, Δsensory FSS, Δcommunication FSS, Δmotor FSS, Δfeeding FSS, and Δrespiratory FSS (F=13.56, 8.97, 10.84, 6.30, 7.37, 7.84, 7.47, 9.97, 10.50, all P<0.05). Conclusion The incidence of new-onset organ dysfunction in PICU was high. The case fatality rate in patients with new-onset organ dysfunction was high. The functional status at hospital discharge was strongly associated with the functional status at admission. Patients in the cardiovascular disease group had the highest incidence of new-onset organ dysfunction and the most severe deterioration of functional status.More attention must be paid to motor function and respiratory function in cardiovascular disease, respiratory disease and hematological disease.

Objective:To investigate the level of endogenous hydrogen sulfide (H 2S) in contrast-induced acute kidney injury (CIAKI), as well as the potential role of H 2S against CIAKI by down-regulating NLRP3 inflammasome. Methods:Twenty-four healthy male Sprague-Dawley rats, weighing 180-220 g, were randomly divided into three groups according to the random number table method: control group, CIAKI group (iopromide 2.9 g/kg) and CIAKI+NaHS group (NaHS 4 mg/kg for three days before 2.9 g/kg iopromide injection). Kidneys were collected for whole-genome sequencing and bioinformatic analysis. HE and PAS staining were used for kidney histological examination. TUNEL assays were applied to detect renal tubular epithelial injury. Expressions of NLRP3 inflammasome (NLRP3, ASC and caspase-1) were evaluated by immunofluorescence staining. The role of H 2S in contrast (iopromide 200 mgI/kg)-induced injury on human renal tubular epithelium (HK-2 cells) was investigated, and CCK-8 assay was used to detect cellular viability. Results:Compared with the control group, the expression of endogenous H 2S synthetases-related genes [cystathionine β-synthase ( CBS), cystathionine-γ-lyase ( CSE) and 3-mercaptopyruvate sulfurtransferase ( 3- MST)] was lower in CIAKI group (all P<0.05). The gene expression levels of CBS, CSE and 3- MST were negatively correlated with renal function biomarkers serum creatinine, blood urea nitrogen and cystatin-C (all P<0.05). Compared with the CIAKI group, CIAKI+NaHS group showed alleviated creatinine, blood urea nitrogen and cystatin-C, improved histological changes, reduced apoptosis. Moreover, the expression levels of NLRP3, ASC and caspase-1 in CIAKI+NaHS group were lower than those in CIAKI group (all P<0.05). In HK-2 cells, compared with the contrast group, the cellular viability was higher in the contrast+NaHS group; reducing endogenous H 2S by CBS inhibitor could enhance contrast-induced cell viability ( P<0.05). Conclusions:Injury of endogenous H 2S system is pivotal to CIAKI pathogenesis. Up-regulation of H 2S ameliorates renal injury of CIAKI rats, which may be related to regulation of NLRP3 inflammasome.

Objective To observe the effect of Chaijin-Huayu decoction combined with endoscopic minimally invasive for gallbladder polyposis and its effect on quality of life.Methods A total of 91 patients with gallbladder polyposis were selected from March 2014 to early March 2017 outpatients and wards of Hebei Provincial Traditional Chinese Medicine Hospital.According to the method of random number table,the paitents were randomly divided into the observation group (n=46) and the control group (n=45).After three courses of treatment,the total effective rate,TCM symptom score and quality of life were observed in both groups,and the postoperative complications of the two groups were compared.Results After three courses of treatment,the total effective rate of the observation group was 91.3％ (42/46),while that of the control group was 88.9％ (40/45).There was no significant difference between the two groups (x2=0.149,P=0.592).In the observation group,the scores of subcardiac pycnosis and belching,right flank distended pain and burp were lower than those of the control group (t were 2.431,3.560 and 2.891 respectively,all P＜0.05).In the SF-36 scale,the scores of mental health,social function,physical pain,physiological function,emotional function,vitality,physiological function and overall health of the observation group were higher than those of the control group (t were 2.342,2.831,2.765,2.545,2.485,2.650,2.582,2.325 respectively,all P＜0.05);The incidence of surgical complications of the observation group was 2.2％ (1/46),while that of the control group was 17.8％ (8/45),and the difference between the two groups was statistically significant (x2=0.149,P＞0.05).Conclusions Chaijin-Huayu decoction combined with minimally invasive choledochoplasty could relieve symptoms,improve quality of life and reduce postoperative complications in patients with gallbladder polyposis.

Objective To observe the correlation of intrapulmonary TREM-1 with endoplasmic reticulum stress in mice with acute lung injury (ALI).Methods Balb/c mice were tracheally injected with lipopolysaccharide(LPS,5 mg/kg) to induce ALI.Real-time PCR and Western blot were used to detect the expressions of TREM-1,CHOP and GRP78.The correlation of TREM-1 with endoplasmic reticulum-related proteins was analyzed.LPS (100 ng/mL) was used to induce inflammation in mouse primary peritoneal macrophages,and expressions of TREM -1,CHOP and GRP78 mRNA were detected by real-time PCR.The effect of TREM-1 activation on the expressions of CHOP and GRP78 was observed in macrophages.Results The expressions of TREM-1,CHOP and GRP78 mRNA were increased in ALI mice.TREM-1 mRNA expression was positively correlated with CHOP and GRP78 mRNA expression.In vitro,LPS up-regulated the expressions of TREM-1,CHOP and GRP78,and TREM-1 was positively correlated with CHOP and GRP78.Activation of TREM-1 increased CHOP and GRP78 mRNA expressions.Conclusions TREM-1 is positively related to the endoplasmic reticulum stress.The activation of TREM-1 enhances endoplasmic reticulum in mouse macrophages.

Chronic Guillain-Barre syndrome, also known as chronic inflammatory demyelinating polyradiculoneuropathy(CIDP), is an immune-mediated demyelinating peripheral neuropathy. This article analyzes the clinical data of a CIDP patient presenting primarily with limb numbness, pain, and weakness. Along with literature review, this study explores the differential diagnosis between CIDP and diabetic peripheral neuropathy in terms of the pathogenesis, clinical manifestations, laboratory tests, and treatment.