Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 12 de 12
Filter
Add filters








Year range
1.
Chinese Journal of Ocular Fundus Diseases ; (6): 197-204, 2022.
Article in Chinese | WPRIM | ID: wpr-934294

ABSTRACT

Objective:To observe the different clinical response patterns of uveal melanoma (UM) patients after external scleral plaque radiotherapy (PRT), and to investigate the risk factors of secondary enucleation after treatment failure.Methods:A single-centre retrospective study. Demographic baseline characteristics and clinical data were collected from 465 UM patients treated with 125I external scleral PRT at Beijing Tongren Hospital from March 2011 to September 2017. Among them, 217 were male and 248 were female, tumor all occurred monocularly. The mean age of subjects was 46.7±12.1 years. Reasons for secondary enucleation included local tumor treatment failure, glaucoma, scleral necrosis and patient request. Tumor grading was based on the grading standards established by the American Joint Committee on Cancer (AJCC). The pattern of tumor response after PRT was classified as degenerated type, growth type, stable type or other types according to literature criteria. The median follow-up time after PRT was 59 months to observe tumor changes. Complete follow-up records of 3 or more color doppler ultrasound imaging (CDI) was available in 245 cases. A t-test was performed to compare the patient's age, intraocular pressure, best corrected visual acuity, tumor thickness and maximum basal diameter before treatment; a chi-square test was performed to compare the patient's gender, AJCC T classification of the tumor, whether the ciliary body was involved, presence of subretinal fluid, optic disc invasion and vitreous hemorrhage, tumor shape and location. Kaplan-Meier survival analysis was used to estimate the cumulative probability of secondary enucleation after extra-scleral PRT. Univariate and multivariate Cox proportional hazards regression analyses were used to evaluate the relationship between tumor characteristics and secondary enucleation after extra-scleral PRT. Results:Among 465 patients, eecondary enucleation was performed on 78 (16.8%, 78/465) patients during the follow-up period. The 1, 3 and 5 year secondary enucleation rates were 5.4%, 9.3% and 17.1 %, respectively. Eye preservation was successful in 387 cases (83.2%, 387/465). Patients treated by secondary enucleation had a larger maximum basal diameter of tumor, a higher proportion of irregular and diffuse morphology, a cumulative macular involved and a higher AJCC T classification, the difference was statistically significant ( P<0.05). There were 115, 76, 27, and 27 cases of degenerated type, stable type, growth type, and other type, respectively. The tumor thickness of the growth type and other types was significantly smaller than that of the degenerated type and the stable type, and the difference was statistically significant ( P<0.05). Univariate Cox analysis showed that the maximum basal diameter of the tumor ( HR=1.19), tumor thickness ( HR=1.08), AJCC T classification ( HR=1.90), growth type response pattern (relative to degenerated type response pattern) ( HR=4.20) was associated with failure of eye preservation ( P<0.05). In the multivariate Cox analysis, the largest tumor basal diameter ( HR=1.24) and the growth type response pattern (relative to the degenerated type response pattern) ( HR=4.59) were still associated with failure of eye preservation ( P<0.05). Conclusions:The tumor thickness of UM patients with growing and other response patterns after PRT is smaller before treatment; the maximum basal diameter of the tumor and the growing response pattern are independent risk factors for secondary enucleation.

2.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1377-1381, 2019.
Article in Chinese | WPRIM | ID: wpr-802938

ABSTRACT

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.

3.
Chinese Journal of Experimental Ophthalmology ; (12): 833-838, 2019.
Article in Chinese | WPRIM | ID: wpr-796595

ABSTRACT

Retinopathy of prematurity (ROP) is a disease which the retinal vascular system is not mature at birth, leading to cessation of development and abnormal proliferation of blood vessels, in a relatively high oxygen environment.Adiponectin (APN) is an endogenous bioactive protein secreted by adipocytes.It has functions of regulating Glucose and lipid metabolism, inhibiting atherosclerosis and protecting the cardiovascular system.APN can inhibit angiogenesis which may be involved in the occurrence and development of ROP in recent research.APN may be regulating the serum level of tumor necrosis factor α (TNF-α), insulin-like growth factor 1 (IGF-1), and vascular endothelial growth factor (VEGF) A to prevent the development of ROP.The expression of APN is also affected by fibroblast growth factor 21 (FGF-21) and long-chain polyunsaturated fatty acids (ω-3 LCPUFAs) at the same time.Understanding the role of APN in ROP can provide new ideas for further study of the pathogenesis and prevention and treatment of ROP.

4.
Chinese Journal of Experimental Ophthalmology ; (12): 833-838, 2019.
Article in Chinese | WPRIM | ID: wpr-790170

ABSTRACT

Retinopathy of prematurity ( ROP) is a disease which the retinal vascular system is not mature at birth,leading to cessation of development and abnormal proliferation of blood vessels, in a relatively high oxygen environment. Adiponectin ( APN ) is an endogenous bioactive protein secreted by adipocytes. It has functions of regulating Glucose and lipid metabolism,inhibiting atherosclerosis and protecting the cardiovascular system. APN can inhibit angiogenesis which may be involved in the occurrence and development of ROP in recent research. APN may be regulating the serum level of tumor necrosis factorα(TNF-α),insulin-like growth factor 1 (IGF-1),and vascular endothelial growth factor ( VEGF) A to prevent the development of ROP. The expression of APN is also affected by fibroblast growth factor 21 (FGF-21) and long-chain polyunsaturated fatty acids (ω-3 LCPUFAs) at the same time. Understanding the role of APN in ROP can provide new ideas for further study of the pathogenesis and prevention and treatment of ROP.

5.
Chinese Journal of Pediatrics ; (12): 451-456, 2017.
Article in Chinese | WPRIM | ID: wpr-808773

ABSTRACT

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.

6.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1393-1397, 2017.
Article in Chinese | WPRIM | ID: wpr-659171

ABSTRACT

Objective To investigate the high risk factors of new dysfunction in Pediatric Intensive Care Unit.Methods A retrospective cohort case control analysis was performed in order to investigate the 906 patients admitted to the Pediatric Intensive Care Unit of Shengjing Hospital of China Medical University from January 2015 to January 2016.Assessment of Functional Status Scale(FSS) was performed at both admission and discharge.According to the new dysfunction diagnostic criteria,all cases were divided into new dysfunction group and control group.The 2 groups were compared in gender,glucocorticoids application time,mechanical ventilation application time,sedation application time,neuromuscular blockers application time,albumin,alanine aminotransferase,aspartate aminotransferase,creatine kinase,creatine kinase isoenzyme,leukocyte,hemoglobin and C-reactive protein.Multivariate Logistic regression was used to screen the risk factors leading to new dysfunction.All cases were divided into high risk factors group and control group according to the risk factors.The 2 groups were compared in initial FSS,FSS of discharge,ΔFSS and the discharge FSS of mental status domain,FSS of sensory domain,FSS of communication domain,FSS of motor domain,FSS of feeding domain,and FSS of respiratory domain.Results Among the 906 cases,547 cases were male and 359 cases were female,and average age was (28.1-± 1.9) months.There were 81 cases in new dysfunction group and 825 cases in control group.Factors such as mechanical ventilation application time [(3.7 ±-0.5) d vs.(1.1 ±-0.1) d],glucocorticoid application time [(3.2-±0.6) d vs.(1.7-±0.1) d],sedation application time[(4.7 ±0.7) d vs.(1.7 ±0.1)d],neuromuscular blockers application time [(0.7 ± 0.3) d vs.(0.1 ± 0.03) d],albumin [(35.6 ± 0.8) g/L vs.(40.5 ± 0.2) g/L],creatine kinase isoenzyme [(75.8 ± 12.4) U/L vs.(49.7 ± 2.6) U/L] had significant differences between the new dysfunction group and the control group(all P < 0.05).Multivariate Logistic regression analysis revealed that more than 7 days of mechanical ventilation,more than 7 days of glucocorticoids application,more than 7 days of sedation application,hypoalbuminemia were risk factors to develop new dysfunction [OR =0.69 (95% CI:0.62-0.78),OR=0.62 (95% CI:0.75-0.94),0R=0.75 (95% CI:0.68-0.84),0R=0.68 (95% CI:1.06-1.16),all P <0.05].In the more than 7 days of mechanical ventilation group,FSS at discharge,FSS of mental status,FSS of sensory,FSS of communication,FSS of motor,FSS of feeding,FSS of respiratory were significantly different from those of the mechanical ventilation application time ≤ 7 d group (all P < 0.05).In the more than 7 days of glucocorticoids application group,FSS of mental status,FSS of sensory,FSS of communication were significantly different from those of the glucocorticoids application time ≤7 d group (all P < 0.05).In the more than 7 days of sedation application group,FSS at discharge,FSS of mental status,FSS of sensory,FSS of communication,FSS of motor were significantly different from those of the sedation application time ≤7 d group(all P < 0.05).Concltsion More than 7 days of mechanical ventilation,glucocorticoids application and sedation application not only increase the incidence of new dysfunction,but also affect mental,sensory,communication,motor function,the muscle and cognitive function at discharge and prognosis.

7.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1393-1397, 2017.
Article in Chinese | WPRIM | ID: wpr-661987

ABSTRACT

Objective To investigate the high risk factors of new dysfunction in Pediatric Intensive Care Unit.Methods A retrospective cohort case control analysis was performed in order to investigate the 906 patients admitted to the Pediatric Intensive Care Unit of Shengjing Hospital of China Medical University from January 2015 to January 2016.Assessment of Functional Status Scale(FSS) was performed at both admission and discharge.According to the new dysfunction diagnostic criteria,all cases were divided into new dysfunction group and control group.The 2 groups were compared in gender,glucocorticoids application time,mechanical ventilation application time,sedation application time,neuromuscular blockers application time,albumin,alanine aminotransferase,aspartate aminotransferase,creatine kinase,creatine kinase isoenzyme,leukocyte,hemoglobin and C-reactive protein.Multivariate Logistic regression was used to screen the risk factors leading to new dysfunction.All cases were divided into high risk factors group and control group according to the risk factors.The 2 groups were compared in initial FSS,FSS of discharge,ΔFSS and the discharge FSS of mental status domain,FSS of sensory domain,FSS of communication domain,FSS of motor domain,FSS of feeding domain,and FSS of respiratory domain.Results Among the 906 cases,547 cases were male and 359 cases were female,and average age was (28.1-± 1.9) months.There were 81 cases in new dysfunction group and 825 cases in control group.Factors such as mechanical ventilation application time [(3.7 ±-0.5) d vs.(1.1 ±-0.1) d],glucocorticoid application time [(3.2-±0.6) d vs.(1.7-±0.1) d],sedation application time[(4.7 ±0.7) d vs.(1.7 ±0.1)d],neuromuscular blockers application time [(0.7 ± 0.3) d vs.(0.1 ± 0.03) d],albumin [(35.6 ± 0.8) g/L vs.(40.5 ± 0.2) g/L],creatine kinase isoenzyme [(75.8 ± 12.4) U/L vs.(49.7 ± 2.6) U/L] had significant differences between the new dysfunction group and the control group(all P < 0.05).Multivariate Logistic regression analysis revealed that more than 7 days of mechanical ventilation,more than 7 days of glucocorticoids application,more than 7 days of sedation application,hypoalbuminemia were risk factors to develop new dysfunction [OR =0.69 (95% CI:0.62-0.78),OR=0.62 (95% CI:0.75-0.94),0R=0.75 (95% CI:0.68-0.84),0R=0.68 (95% CI:1.06-1.16),all P <0.05].In the more than 7 days of mechanical ventilation group,FSS at discharge,FSS of mental status,FSS of sensory,FSS of communication,FSS of motor,FSS of feeding,FSS of respiratory were significantly different from those of the mechanical ventilation application time ≤ 7 d group (all P < 0.05).In the more than 7 days of glucocorticoids application group,FSS of mental status,FSS of sensory,FSS of communication were significantly different from those of the glucocorticoids application time ≤7 d group (all P < 0.05).In the more than 7 days of sedation application group,FSS at discharge,FSS of mental status,FSS of sensory,FSS of communication,FSS of motor were significantly different from those of the sedation application time ≤7 d group(all P < 0.05).Concltsion More than 7 days of mechanical ventilation,glucocorticoids application and sedation application not only increase the incidence of new dysfunction,but also affect mental,sensory,communication,motor function,the muscle and cognitive function at discharge and prognosis.

8.
Chinese Pediatric Emergency Medicine ; (12): 355-359, 2017.
Article in Chinese | WPRIM | ID: wpr-618833

ABSTRACT

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.

9.
Chinese Pediatric Emergency Medicine ; (12): 450-454, 2016.
Article in Chinese | WPRIM | ID: wpr-501947

ABSTRACT

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.

10.
Chinese Pediatric Emergency Medicine ; (12): 37-40, 2016.
Article in Chinese | WPRIM | ID: wpr-491537

ABSTRACT

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.

11.
Chinese Pediatric Emergency Medicine ; (12): 304-308, 2015.
Article in Chinese | WPRIM | ID: wpr-463569

ABSTRACT

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.

12.
Chinese Pediatric Emergency Medicine ; (12): 84-88, 2014.
Article in Chinese | WPRIM | ID: wpr-445154

ABSTRACT

Neuromuscular blocking agents have been used for a variety of clinical conditions such as emergency intubation,acute respiratory distress syndrome,status asthmaticus,elevated intracranial pressure,etc.Neuromuscular blocking agents should be used and monitored appropriately according to evidence-based guidelines to reduce the adverse events associated with the use of neuromuscular blocking agents.

SELECTION OF CITATIONS
SEARCH DETAIL