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1.
Chinese Journal of Neonatology ; (6): 327-331, 2023.
Article in Chinese | WPRIM | ID: wpr-990758

ABSTRACT

Objective:To study the risk factors and clinical outcomes of early pulmonary hypertension in preterm infants with gestational age(GA)≤32 w.Methods:From October 2017 to May 2021,preterm infants with GA≤ 32 w admitted to NICU of our hospital were retrospectively studied. According to their echocardiography 2 w after birth, the infants were assigned into early-onset pulmonary hypertension (ePH) group and non-PH group. SPSS 21.0 statistical software was used to analyze the general status, complications and clinical outcomes of the two groups. Multiple logistic regression was used to analyze the risk factors of early-onset PH.Results:A total of 183 cases were enrolled, including 24 in the ePH group and 159 in the non-PH group. The incidences of birth asphyxia, hemodynamically significant patent ductus arteriosus (hsPDA), FiO 2≥30% within 6 h after birth, late-onset PH, severe bronchopulmonary dysplasia(BPD) and intracranial hemorrhage(ICH) in the ePH group were significantly higher than the non-PH group( P<0.05). hsPDA was the independent risk factor for early-onset PH ( OR=11.781, 95% CI 4.192-33.108). Conclusions:Preterm infants with GA≤32 w and early-onset PH are at increased risks of ICH, late-onset PH and severe BPD, hsPDA is the independent risk factor for early-onset PH.

2.
Chinese Pediatric Emergency Medicine ; (12): 364-367, 2023.
Article in Chinese | WPRIM | ID: wpr-990529

ABSTRACT

Objective:To investigate the clinical characteristics of congenital esophageal atresia with gastric perforation, and to improve pediatricians′ understanding of this disease.Methods:The clinical data of five neonates with congenital esophageal atresia and gastric perforation treated in the neonatal intensive care unit of the Affiliated Hospital of Qingdao University from 2012 to 2022 were analyzed retrospectively.Results:Among the five neonates, four were boys and one was girl.The gestational age was 28 + 5 to 37 + 6 weeks, the birth weight was 1 100~2 350 g. All of them had dyspnea and feeding difficulties after birth.Gastric perforation occurred in three cases during invasive mechanical ventilation, one case during non-invasive ventilation, and one case during nasal catheter oxygen inhalation.Emergency primary gastric repair was performed, followed by secondary esophageal anastomosis.All the patients were cured and discharged from hospital. Conclusion:Gastric perforation is a rare complication of congenital esophageal atresia, which is more common in premature infants and low birth weight infants.Mechanical ventilation may promote the occurrence of gastric perforation.If gastric perforation is complicated, repair should be performed as soon as possible, and esophageal anastomosis surgery should be performed early after stability to improve the final outcome.

3.
Chinese Journal of Practical Nursing ; (36): 241-247, 2023.
Article in Chinese | WPRIM | ID: wpr-990167

ABSTRACT

Objective:To investigate the intervention effect of decision aid on the fear of falling in elderly patients after total hip arthroplasty.Methods:This study was a quasi experimental research. From June 2021 to November 2021, 84 patients after total hip arthroplasty who were admitted to the Department of Orthopedics of Zhengzhou Central Hospital Affiliated to Zhengzhou University were selected as the research objects. According to the order of admission, they were divided into the control group (42 cases, 2 cases fell off ) and the observation group (42 cases). The control group was given routine nursing care and guidance for fear of falling, while the observation group was given a decision-making aid intervention program on the basis of the control group. The time to first getting out of bed, the scores of Modified Falls Efficacy Scale (MFES), Generalized Anxiety Disorder (GAD-7) and Harris Hip Score (HHS) were compared between two groups.Results:The time to get out of bed for the first time in the observation group was (42.71 ± 6.41) h, lower than that in the control group (49.95 ± 5.73) h, and the difference was statistically significant ( t=5.38, P<0.05). Twelve weeks after discharge, the GAD-7 score in the observation group was (4.64 ± 1.43), which was lower than that of the control group (6.85 ± 1.83), and the difference was statistically significant ( t=6.10, P<0.05). The score of the MFES in the observation group was [8.50(8.00,10.00)], which was higher than that in the control group [7.50(7.00,8.00)], and the difference was statistically significant ( Z=-6.26, P<0.05). The hip joint function score of the observation group was (81.74 ± 4.24), which was higher than that of the control group (74.30 ± 4.51), and the difference was statistically significant ( t=7.69, P<0.05). Conclusions:Decision support can advance the time of downward movement of elderly patients after total hip arthroplasty, reduce their anxiety and fear of falling, and improve hip function.

4.
Chinese Journal of Neonatology ; (6): 143-146, 2022.
Article in Chinese | WPRIM | ID: wpr-931005

ABSTRACT

Objective:To study the effects of different sizes of maternal placental chorionic hemangioma (PCH) on neonatal clinical outcome.Methods:February 2013 to December 2020, neonates whose mothers with PCH delivered in our hospital were retrospectively analyzed. According to the diameter of PCH, the neonates were assigned into giant PCH group (diameter≥4 cm) and ordinary PCH group (diameter<4 cm). Clinical characteristics and outcomes were compared between the two groups.Results:A total of 35 cases were enrolled in the study. 13 cases (37.1%) were male, 12 cases (34.3%) were Cesarean section delivered, 11 cases (31.4%) were premature infants, 12 cases (34.3%) had low birth weight and 12 cases (34.3%) were admitted to NICU, 7 cases (20.0%) had intrauterine distress, cardiac enlargement and abnormal hematological indexes, respectively, 6 cases (17.1%) needed respiratory support; 5 cases (14.2%) had increased amniotic fluid and fetal edema, respectively, 4 cases (11.4%) received blood transfusion, 3 cases (8.5%) had postnatal asphyxia, 2 cases (5.7%) had brain injury and 2 cases (5.7%) had congenital malformation. 15 cases were in the giant PCH group and 20 cases in the ordinary PCH group. Compared with the ordinary PCH group, the giant PCH group had significantly higher incidences of prematurity, low birth weight, increased amniotic fluid, intrauterine distress, NICU hospitalization, fetal edema, cardiac enlargement, respiratory support, abnormal hematological indexes, blood transfusion and mortality ( P<0.05). Conclusions:Maternal complications with giant PCH may significantly increase the risk of neonatal complications, thus perinatal monitoring should be strengthened.【 Key words】Placental chorionic hemangioma; Infant, newborn; Clinical outcome

5.
Chinese Journal of Clinical Nutrition ; (6): 148-156, 2021.
Article in Chinese | WPRIM | ID: wpr-909336

ABSTRACT

Objective:To investigate the effects of nutritional intake in the first two weeks of life on bronchopulmonary dysplasia (BPD) in preterm infants with gestational age (GA) ≤ 32 weeks.Methods:A retrospective case-control study was conducted 154 preterm infants with birth weight ≤ 1500 g and GA ≤ 32 weeks were enrolled from neonatal intensive care unit (NICU) of Affiliated Hospital of Qingdao University between January 1, 2016 and December 31, 2017. These infants were divided into BPD group or non-BPD group. All clinical and nutritional data were collected and analyzed to investigate the effects of early-life (within 2 weeks after birth) nutritional intake on BPD.Results:Among a total of 154 eligible neonates, 68 were without BPD and 86 with BPD (55.8%). Mild, moderate and severe BPD accounted for 39.5% (34/86), 58.1%(50/86)and 2.4%(2/86)of all BPD cases respectively. GA and birth-weight of BPD group were significantly lower than that of non-BPD group [(28.35 ± 1.55)weeks vs. (30.12 ± 1.23)weeks; (1050.91 ± 190.6)g vs. (1205.88 ± 195.83)g, both P = 0.000]. The duration of mechanical ventilation in BPD group was longer than that in non-BPD group [(2.65 ± 1.08)days vs. (0.47 ± 0.12)days, P < 0.05]. The incidences of complications in BPD group, including neonatal asphyxia, sepsis and patent ductus arteriosus, were all higher than those in non-BPD group( P < 0.05). The fluids and caloric intake, enteral fluids and caloric intake were significantly lower in BPD group on Day 7 and 14 of life ( P < 0.05). The macronutrient intake in BPD group was also consistently lower, reaching statistical significance for carbohydrate intake on Day 7 and 14 of life, and for protein and lipid intake on Day 14 of life ( P < 0.05). Multivariate logistic regression analysis showed that mechanical ventilation ( OR = 2.257, 95% CI: 1.143~4.456, P = 0.019) and GA ( OR = 0.325, 95% CI: 0.215~0.49, P = 0.000) were high-risk factors for BPD. The decreased odds of developing BPD were associated with higher levels of enteral calories on Day 14 of life ( OR = 0.96, 95% CI: 0.94~0.98, P = 0.000), fluids on Day 7 of life ( OR = 0.927, 95% CI: 0.876~0.981, P = 0.009) and protein intake on Day 14 of life ( OR = 0.044, 95% CI: 0.011~0.177, P = 0.000). Conclusions:GA and mechanical ventilation were independent high-risk factors for BPD. Higher intake of protein and enteral calories were protective factors. Proactive early enteral nutrition support, adequate protein intake and decreasing the duration of mechanical ventilation may reduce the risk of BPD.

6.
Chinese Journal of Clinical Nutrition ; (6): 374-380, 2019.
Article in Chinese | WPRIM | ID: wpr-824191

ABSTRACT

Objective To explore the high-risk factors of metabolic bone disease (MBD) in premature infants by retrospective analysis of the clinical data so as to provide evidence for optimal clinical management. Methods Clinical data of premature infants with birth weight<1500 g admitted in our hospital from January 2016 to December 2017 were retrospectively analyzed. Infants with serum alkaline phosphatase ( ALP )>500 IU/L and blood phosphorus <1. 5 mmol/L were selected as MBD group and premature infants with birth weight <1500 g were selected randomly as non-MBD group. General data, pulmonary surfactant, continuous positive airway pressure, mechanical ventilation, start time of enteral nutrition, parenteral nutrition ( PN) time, breast feeding time and breast milk fortifier adding, drug usage, hospitalization time and complications were re-corded and compared between the two groups. Results A total of 440 premature infants with birth weight<1500 g were admitted to the hospital during the study period. 58 [ 13. 2% ( 58/440) ] infants were enrolled in the MBD group, among which infants with birth weight<1000 g accounting for 56. 9% ( 33/58) . High birth weight (OR=0. 62, 95% CI:0. 389-0. 990) was an independent protective factor of MBD in premature in-fants. The higher the birth weight, the lower the risk of MBD in premature infants. The longer duration of breast feeding time ( OR= 2. 191, 95% CI:1. 628-2. 950) , later initial time of enteral feeding ( OR=2. 695, 95%CI:1. 710-4. 248), longer duration of PN (OR=6. 205, 95% CI:3. 359-11. 463) time, longer duration of respiratory supporting time ( OR=1. 046, 95% CI:1. 026-. 067) , longer hospital stay time ( OR=1. 703, 95% CI:1. 109-2. 615) and small for gestational age ( OR=2. 965, 95% CI:1. 163-5. 658) were inde-pendent risk factors of MBD in premature infants. The duration of PN was the most important independent risk factor of MBD in premature infants (OR=6.205, 95% CI: 3.359-11.463). Conclusion Multiple factors can lead to MBD of premature infants. The high birth weight is an independent protective factor of MBD and the duration of PN is the most important independent risk factor of MBD in premature infants.

7.
Chinese Journal of Applied Clinical Pediatrics ; (24): 542-545, 2019.
Article in Chinese | WPRIM | ID: wpr-743509

ABSTRACT

Objective To investigate the mechanism of white matter damage (WMD) and the neuroprotective effect of Xenon on neonates with WMD.Methods Three-day-old SD rat pups (n =96) were randomly divided into the blank control group (n =24),the WMD control group (n =24),the Xenon intervention group A (n =24) and the Xenon intervention group B (n =24) by random number method according to their birth time.WMD rat models were successfully established by giving intraperitoneal injection of lipopolysaccharide(LPS) 0.05 mg/kg combined with carotid artery ligation and hypoxia for 1 hour in the WMD control group and the Xenon intervention groups.In the control group,only 9 g/L saline (0.05 mg/kg) was injected intraperitoneally,while carotid artery ligation and hypoxia were not administered.Rats in Xenon intervention group A and group B were given inhalation of 500 mL/L Xenon for 3 hours at 0 and 2 hours respectively after establishment of the models.Six rats in each group were randomly selected and decapitated at 0,24,48 and 72 hours after the intervention.The brain white matter on the right was analyzed by using HE staining and myelin basic protein(MBP) immunofluorescence staining,and real-time quantitative polymerase chain reaction was used to detect the expressions level of CLIC4 mRNA.Results (1) Brain tissue pathology:compared with the blank control group,the brain white matter on the right of the WMD control group and the Xenon intervention group A and group B had loose and disordered structure,nuclear pyknosis and cytoplasm loosening.However,the lesions in both Xenon intervention group A and group B were significantly less than those in the WMD control group,and there was no significant difference between the Xenon intervention group A and group B.(2) MBP measurement:the number of MBP-positive cells in the brain white matter on the right of WMD control group was significantly lower than that in the blank control group,while compared with WMD control group,they were significantly higher in Xenon intervention group A and group B.(3) CLIC4 mRNA expression level:compared with blank control group,the expressions levels of CLIC4 mRNA at most time point were higher both in the WMD control group and the Xenon intervention group A and group B (all P < 0.05),except the time point 24 h in the Xenon intervention group A.The expressions of CLIC4 mRNA in group A and group B were significantly decreased compared with those in the WMD control group (all P < 0.05).However,there were no significant differences between Xenon intervention group A and group B (P > 0.05).Conclusions The expressions of CLIC4 mRNA in brain tissues on neonatal rats with WMD significantly increased,indicating that the mitochondrial pathway could be one of the pathological processes of WMD.Early Xenon intervention may reduce neonatal WMD by reducing the expression of CLIC4 mRNA,which plays a neuroprotective role.

8.
Chinese Journal of Neonatology ; (6): 214-218, 2018.
Article in Chinese | WPRIM | ID: wpr-699294

ABSTRACT

Objective To investigate the pathogenesis of white matter damage (WMD) and the effects of xenon intervention on the expression of EphB4 and EphrinB2 mRNA in the brain tissue of neonatal rats.Method Three-day-old SD rat pups (n =96) were randomly assigned into sham group (n =24),model group (n =24),xenon intervention group 1 (n =24) and xenon intervention group 2 (n =24).The WMD model was established by injected of lipopolysaccharide (LPS) 0.05 mg/kg combined with ligation of the right carotid artery for 1 h in the last three groups.Rats in xenon intervention group 1 inhaled 50% xenon immediately for 3 h after modeling,while rats in xenon intervention group 2 inhaled 50% xenon for 3 h at 2 h after modeling.After the completion of xenon intervention,6 rat pups in each groups were sacrificed at 0 h,24 h,48 h and 72 h.The pathologic examination of periventricular tissue was conducted with hematoxylin-eosin staining (HE) and the expression of EphB4 and EphrinB2 mRNA was assayed by real-time quantitative polymerase chain reaction (RT-PCR).Statistical analysis was then performed.Result (1)The structure of white matter in model group became loose,band net-like,with significant nucleus pyknosis.The pathological damages in xenon intervention group 1 and 2 were lighter at 24 h,48 h and 72 h than model group,with less karyopycnosis.(2) Compared with the sham group,the expressions of EphB4 and EphrinB2 mRNA at 0 h,24 h,48 h and 72 h were significantly higher in the model group and xenon intervention group 1 and 2 (P < 0.05),except for the EphB4 mRNA in xenon intervention group 1 at 72 h (P > 0.05).The expressions of EphB4 and EphrinB2 mRNA at each time point in xenon intervention group 1 and 2 were decreased significantly than the model group (P < 0.05),except for the EphB4 mRNA in xenon intervention group 2 at 72 h (P > 0.05).However,there was no statistically significant difference on EphB4 and EphrinB2 mRNA between two xenon intervention groups at each time point (P > 0.05).Conclusion The expression of EphB4 and EphrinB2 mRNA are appreciably increased in brain tissue of neonatal rats with WMD,which indicates the reactive angiogenesis.The intervention with xenon may play a neuroprotective role through reducing the expressions of EphB4/EphrinB2 mRNA and angiogenesis,and early intervention may be better.

9.
Chongqing Medicine ; (36): 1444-1448, 2018.
Article in Chinese | WPRIM | ID: wpr-691968

ABSTRACT

Objective TGF-β1 can promote EMT,then strengthen the invasion and metastasis ability of cancer ceils.However,the mechanism for TGF-β1 in gastric cancer still keeps unclear.Aim of this study was to investigate the expression of epithelial to mesenchymal transition (EMT)marker,LMO1 and metastasis related genes on the human gastric cancer cell cell line MKN28 treated with TGF-β1,and test whether down-regulate LMO1 expression can affect the pro-EMT and pro-metastatic roles of TGF-β1 in MKN28 cells.Methods Primary human gastric cancer cell line MKN28 was cultured in vitro.Cells were treated with TGF-β1 to induce cells to undergone EMT.Cells were divided into four groups:control group (5 % BSA),TGF-β1 induced group (10 μg/L),negative transfect group (TGF-β1 +negative transfect siRNA),and LMO1-siRNA transfect group (TGF-β1+ LMO1-siRNA).Real time-PCR and Western blot was used to examine the difference of EMT marker (E-cadherin and N-cadherin),LMO1 and metastasis related genes (MMP-9 and VEGF)expression.Transwell assays were performed to identify the differences and changes of invasive and metastatic ability in gastric cancer cell line MKN28.Western blot was used to examine the expression levels of MMP-9 and VEGF.Results TGF-β1 stimulation induced classical EMT morphological change,as was confirmed by E-cadherin decrease and N-cadherin,LMO1,MMP-9,VEGF increase (P<0.01).Accompanied with the EMT,cell invasion and migration ability was markedly increased (P<0.01).However,Down-regulation of LMO1 expression reversed the pro-migratory effect of TGF-β1 to a great degree (P<0.01).Conclusion LMO1 played a central role in coordinating TGF-β1 induced EMT and pro-migratory effects in gastric cancer MKN28 cells.Using siRNA to downregulate the expression of LMO1 can inhibit the invasion and metastasis ability of gastric cancer MKN28 cells.

10.
Protein & Cell ; (12): 590-600, 2017.
Article in English | WPRIM | ID: wpr-756983

ABSTRACT

Entero virus 71 (EV71) causes hand, foot, and mouth disease (HFMD) and occasionally leads to severe neurological complications and even death. Scavenger receptor class B member 2 (SCARB2) is a functional receptor for EV71, that mediates viral attachment, internalization, and uncoating. However, the exact binding site of EV71 on SCARB2 is unknown. In this study, we generated a monoclonal antibody (mAb) that binds to human but not mouse SCARB2. It is named JL2, and it can effectively inhibit EV71 infection of target cells. Using a set of chimeras of human and mouse SCARB2, we identified that the region containing residues 77-113 of human SCARB2 contributes significantly to JL2 binding. The structure of the SCARB2-JL2 complex revealed that JL2 binds to the apical region of SCARB2 involving α-helices 2, 5, and 14. Our results provide new insights into the potential binding sites for EV71 on SCARB2 and the molecular mechanism of EV71 entry.


Subject(s)
Animals , Humans , Mice , Amino Acid Sequence , Antibodies, Monoclonal , Chemistry , Genetics , Metabolism , Binding Sites , Cell Line , Crystallography, X-Ray , Enterovirus A, Human , Genetics , Allergy and Immunology , Fibroblasts , Virology , Gene Expression , HEK293 Cells , Immunoglobulin Fab Fragments , Chemistry , Genetics , Metabolism , Lysosome-Associated Membrane Glycoproteins , Chemistry , Genetics , Allergy and Immunology , Models, Molecular , Protein Binding , Protein Conformation, alpha-Helical , Protein Conformation, beta-Strand , Protein Interaction Domains and Motifs , Receptors, Scavenger , Chemistry , Genetics , Allergy and Immunology , Receptors, Virus , Chemistry , Genetics , Allergy and Immunology , Recombinant Fusion Proteins , Chemistry , Genetics , Allergy and Immunology , Sequence Alignment , Sequence Homology, Amino Acid , Sf9 Cells , Spodoptera , Thermodynamics
11.
Chinese Journal of General Surgery ; (12): 843-846, 2016.
Article in Chinese | WPRIM | ID: wpr-502047

ABSTRACT

Objective To investigate the expression level of LMO1 in gastric cancer tissues and human gastric cancer cell lines,and explore the invasive and metastatic potential of LMO1 gene silencing by small interfering RNA on the human gastric cancer cell line MKN28.Methods Immunohistochemical technique was applied to detect the expression of LOM1 protein in gastric cancer tissues and tumor adjacent tissues of paraffin specimens in 30 cases.The expression levels of LMO1 in human gastric cancer cell lines AGS,BGC-823,SGC-7901,MKN28 and human gastric mucosal epithelial cells GES were detected by realtime-PCR and Western blot.Using LipofectamineTM 2000,LMO1 siRNA was transfected into MKN28 cellsin vitro (siRNA transfect group).Negative control was established.Real time-PCR and Western blot was used to examine the difference of LMO1 expression.Transwell assays were performed to identify the differences and changes of invasive and metastatic ability in gastric cancer cell line MKN28.Western blot was used to examine the expression levels of E-cadherin,MMP-9 and VEGF.Results Positive rate of LOM1 protein in gastric cancer tissues(77%)was higher than that in tumor adjacent tissues (17%) (x2 =21.70,P < 0.01).Positive rate of Vavl protein was higher in lymphatic metastasis group than in non-lymphatic metastasis group(x2 =5.83,P =0.02).Compared with GES,the expression level of LMO1 increased significantly in gastric cancer cell lines,especially in MKN28 (P < 0.01).The expression levels of LMO1 mRNA and protein in LMO1-siRNA transfected MKN28 cells were lower than the matched negative control cells (P <0.01).The invasive and metastatic potentials of LMO1-siRNA transfected MKN28 cellssignificantly decreased (t =-11.53,P <0.01;t =-10.68,P <0.01).The expression levels of E-cadherin were higher than the matched negative control cells;and MMP-9,VEGF protein in LMO1-siRNA transfected MKN28 cells were lower than the matched negative control cells (P < 0.01).Conclusions LMO1 has higher expression level in gastric cancer tissues and some gastric cancer cell lines,and down-regulation of LMO1 can inhibit the invasion and metastasis ability of gastric cancer.

12.
Protein & Cell ; (12): 297-306, 2015.
Article in English | WPRIM | ID: wpr-757590

ABSTRACT

Dendritic cells (DCs) comprise two functionally distinct subsets: plasmacytoid DCs (pDCs) and myeloid DCs (mDCs). pDCs are specialized in rapid and massive secretion of type I interferon (IFN-I) in response to nucleic acids through Toll like receptor (TLR)-7 or TLR-9. In this report, we characterized a CD56(+) DC population that express typical pDC markers including CD123 and BDCA2 but produce much less IFN-I comparing with pDCs. In addition, CD56(+) DCs cluster together with mDCs but not pDCs by genome-wide transcriptional profiling. Accordingly, CD56(+) DCs functionally resemble mDCs by producing IL-12 upon TLR4 stimulation and priming naïve T cells without prior activation. These data suggest that the CD56(+) DCs represent a novel mDC subset mixed with some pDC features. A CD4(+)CD56(+) hematological malignancy was classified as blastic plasmacytoid dendritic cell neoplasm (BPDCN) due to its expression of characteristic molecules of pDCs. However, we demonstrated that BPDCN is closer to CD56(+) DCs than pDCs by global gene-expression profiling. Thus, we propose that the CD4(+)CD56(+) neoplasm may be a tumor counterpart of CD56(+) mDCs but not pDCs.


Subject(s)
Humans , Biomarkers , Metabolism , CD56 Antigen , Genetics , Allergy and Immunology , Cell Lineage , Genetics , Allergy and Immunology , Dendritic Cells , Allergy and Immunology , Metabolism , Pathology , Gene Expression , Hematologic Neoplasms , Genetics , Allergy and Immunology , Pathology , Immunophenotyping , Interferon Type I , Metabolism , Interleukin-12 , Metabolism , Interleukin-3 Receptor alpha Subunit , Genetics , Allergy and Immunology , Lectins, C-Type , Genetics , Allergy and Immunology , Membrane Glycoproteins , Genetics , Allergy and Immunology , Myeloid Cells , Allergy and Immunology , Metabolism , Pathology , Receptors, Immunologic , Genetics , Allergy and Immunology , Terminology as Topic , Toll-Like Receptor 4 , Genetics , Allergy and Immunology , Toll-Like Receptor 7 , Genetics , Allergy and Immunology , Toll-Like Receptor 9 , Genetics , Allergy and Immunology
13.
International Journal of Pediatrics ; (6): 509-512, 2011.
Article in Chinese | WPRIM | ID: wpr-421391

ABSTRACT

Encephalopathy secondary to hereditary metabolic diseases leads to high mortality due to the difficulties in early diagnosis and timely treatment.Survivors are often left with severe sequelaes of the nervous system. A considerable number of hereditary metabolic diseases are not thoroughly understood, but pre-clinical or early-clinical diagnosis and treatment can be attempted with MRI which can generally optimize the outcome.

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