2.A case with type I Crigler-Najjar syndrome.
Shao-Han NONG ; Yan-ming XIE ; Guan-rong CHEN ; Bi-tao ZHANG
Chinese Journal of Pediatrics 2003;41(5):382-382
Crigler-Najjar Syndrome
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complications
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diagnosis
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Female
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Humans
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Infant
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Jaundice
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etiology
3.Evaluation of Diagnosis and Preoperative Management of 53 Cases with Interrupted Aortic Arch in Infancy
yu-mei, LIU ; shao-ru, HE ; yun-xia, SUN ; jian, ZHUANG ; yu-hui, YU ; min-quan, ZHONG ; jin, ZHONG ; sui-xin, LIANG ; shao-han, NONG ; wei, PAN ; mei-ping, HUANG
Journal of Applied Clinical Pediatrics 2006;0(24):-
Objective To evaluate diagnosis and preoperative management of cases with interrupted aortic arch(IAA)in infancy.Methods Fifty-three infants who were admitted to our hospital from Jan.2001 to Nov.2007 were involved.Clinical data,findings of echocardiogram(Echo),spiral CT,MRI,angiocardiography,preoperative management,surgical repair and postoperative outcome were analyzed retrospectively.Results There were 38 boys and 15 girls,weighted 2.18-10.0(4.32?1.60)kg,aged 1 day to 12(3.05?3.53)months,of which 50.94% were neonates.Symptoms at presentation were 90.57% with tachypnea and 83.02% with difficulty in feeding.Eighty three point zero two percentage cases had different degree of congestive heart failure,37.74% of which were in grade Ⅲ heart function.All cases had weakened femoral pulse.All cases were performed Echo,38 cases of them diagnosed as IAA,6 cases as IAA or severe coarctation of aorta(CoA);they were diagnosed as IAA by CT,and 9 as severe CoA who were diagnosed as IAA via CT or operation.Thirty-three cases were performed CT,of which 15 underwent surgical repair,cardiovascular abnormalities revealed by CT were the same as those in surgical findings.Three cases were taken MRI,and 7 cases were performed angiocardiography.According to the results of Echo,CT,MRI,angiocardiography and surgical findings,35 cases were type A,15 cases were type B and 3 cases were type C.Preoperative treatment included maintaining patent ductus areriosis,management of heart failure and supportive treatment.After proper preoperative management of medication,most cases with congestive heart failure were improved.Twenty-six cases underwent surgical repair,16 survived,10 died du-ring perioperative stage.Main cause of death was severe low cardiac output.Conclusions Value of Echo in diagnosis of IAA is limi-ted.Combination of Echo with CT or MRI is a convenient and safe way to diagnose IAA,it can replace the traditional method of Echo combined with angiocardiography.Proper preoperative management is helpful to patients with IAA to pass to surgical repair,and makes for successful operation.
4.Effects of different arterial oxygen partial pressures on serum protein S100β and neuron specific enolase during cardiopulmonary bypass in infants with cyanotic congenital heart disease.
Can HUANG ; Shao-han NONG ; Ji-mei CHEN ; Shao-ru HE ; Ping CHEN ; Yi-qun DING ; Jian-zheng CEN ; Gang XU
Chinese Journal of Pediatrics 2012;50(2):121-125
OBJECTIVEA prospective study was conducted to probe into the relationship between arterial oxygen partial pressure (PaO2) and brain injury during cardiopulmonary bypass (CPB) in infants with cyanotic congenital heart disease (CHD).
METHODEnrolled in the study were 45 cyanotic infants, who were less than three years old and underwent corrective cardiac surgery from August 1(st), 2010 to January 31(st), 2011 at Guangdong General Hospital. All the infants had a pulse oxygen saturation (SpO2) lower than 85% and were randomly allocated into three groups by a specific computer program. In controlled group 1 (G1 group), PaO2 levels were controlled at 80 - 120 mm Hg (1 mm Hg = 0.133 kPa) during CPB; in controlled group 2 (G2 group), PaO2 levels at 120 - 200 mm Hg during CPB; while in uncontrolled group (G3 group), PaO2 levels were at 200 - 400 mm Hg during CPB. Blood samples were collected just before starting CPB, at the end of CPB, and at 3 h, 5 h, and 24 h after CPB (T1, T2, T3, T4, T5) for the determination of serum concentrations of protein S100β, neuron specific enolase (NSE), and adrenomedullin (ADM) by ELISA.
RESULTProtein S100β rose significantly after starting CPB. In group G3, it reached a peak of (699 ± 139) ng/L by the end of CPB, significantly higher than those in groups G1 and G2 [(528 ± 163) ng/L and (585 ± 155) ng/L], and was positively correlated with PaO2 levels (r = 0.526, P < 0.01). NSE levels of group G1 were continuously rising after starting CPB and reached significantly high levels at 3 h or 5 h after CPB [(12.2 ± 3.4) µg/L and (12.3 ± 3.7) µg/L], while those of group G2 rose significantly during CPB [(10.9 ± 4.8) µg/L] and even higher at 3 h or 5 h after CPB [(12.6 ± 5.1) µg/L and (13.2 ± 5.4) µg/L]. NSE levels of group G3 rose significantly during CPB and maintained at a high level [(12.2 ± 5.7) µg/L] afterwards. There was no significant difference in serum ADM concentrations among different time points in each group and among these three groups. All the infants were discharged from the hospital without any obvious nervous symptom and sign.
CONCLUSIONHigh PaO2 during CPB in infants with CHD might cause an increase of serum protein S100β and NSE, indicating that brain injury might become worse with a higher PaO2 and might be positively correlated with PaO2 during CPB.
Cardiopulmonary Bypass ; Child, Preschool ; Cyanosis ; Female ; Heart Defects, Congenital ; blood ; physiopathology ; surgery ; Humans ; Infant ; Male ; Nerve Growth Factors ; blood ; Oximetry ; Oxygen ; blood ; Partial Pressure ; Phosphopyruvate Hydratase ; blood ; Prospective Studies ; S100 Calcium Binding Protein beta Subunit ; S100 Proteins ; blood ; Serum
5.Expression of killer cell inhibitor receptors on immunocompetent cells with relation to graft-versus-host disease after hematopoietic stem cell transplantation.
Lian-Ning DUAN ; Chun CHEN ; Shao-Liang HUANG ; Jian-Pei FANG ; Jing WEI ; Rong BAO ; Yan LI ; Hong-Xing HAN ; Shu-Nong LI
Journal of Experimental Hematology 2003;11(6):625-632
The study was aimed at the exploration of relationship between T cells expressing killer cell inhibitor receptors (KIR, CD158 and CD94) and graft-versus-host disease (GVHD) after hematopoietic stem cell transplantation. The expression rates of CD158a, CD158b and CD94 on T cells and NK cell were detected by flow cytometry and donor/recipient HLA-Cw was analyzed using PCR after peripheral blood stem cell transplantation (PBSCT) and umbilical cord blood transplantation (UCBT). After both PBSCT and UCBT, the rates of CD3(+)CD158a(+) and CD3(+)CD158b(+) T cells increased, especially the rate of CD8(+)CD158b(+) T cells. In both acute and chronic GVHD groups, the rate of CD3(+)CD158b(+) T cells increased, especially in acute GVHD. The CD94 mainly expressed on CD3(+)CD8(+) T cells. The percentage of the expression of CD94 on CD4(+) and CD8(+) cells after UCBT and PBSCT increased significantly. The expression of KIR in GVHD (early stage of transplantation) increased but the expression of KIR in chronic GVHD (advanced stage of transplantation) decreased. Five patients who HLA-Cw matched had no severe GVHD. In four patients who underwent allo-PBSCT and UCBT from related HLA-matched donors, only 2 patients had no aGVHD. Four patients underwent transplantation from unrelated HLA-matched donors had GVHD. These observations suggested that there is some relationship between GVHD and KIR expression on T cells. CD158b might be an inhibitory molecule of T cell activated at early stage after transplantation. Understanding the mechanism of GVHD with the expression of KIR on T cells, especially those binding the HLA-Cw might shed light on the establishment of the specific immunotolerance for the prevention of GVHD. To pay attention to HLA-Cw typing is very important to reduce GVHD and increase GVL effect in related or unrelated HLA-matched transplantation.
Antigens, CD
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analysis
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Antigens, Differentiation, T-Lymphocyte
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analysis
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Genotype
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Graft vs Host Disease
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etiology
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HLA-C Antigens
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genetics
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Hematopoietic Stem Cell Transplantation
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Humans
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Lectins, C-Type
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Receptors, Immunologic
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analysis
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Receptors, Interleukin-2
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analysis
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Receptors, KIR
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Receptors, KIR2DL1
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Receptors, KIR2DL3
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T-Lymphocytes
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immunology
6.Preventive early intervention strategies for neurodevelopmental disorders of high-risk infants
Shao-Han NONG ; Wei-Hong YU ; Cui-Hong LI ; Xiao-Guang ZHOU
Chinese Journal of Contemporary Pediatrics 2024;26(3):297-301
Neurodevelopmental disorders in children have become a significant global public health concern,impacting child health worldwide.In China,the current intervention model for high-risk infants involves early diagnosis and early treatment.However,in recent years,overseas studies have explored novel preventive early intervention strategies for neurodevelopmental disorders in high-risk infants,achieving promising results.This article provides a comprehensive review of the optimal timing,methods,and intervention models of the preventive early intervention strategies for neurodevelopmental disorders in high-risk infants.The aim is to enhance the awareness and knowledge of healthcare professionals regarding preventive early intervention strategies for neurodevelopmental disorders in high-risk infants,facilitate clinical research and application of such interventions in China,and ultimately reduce the incidence of neurodevelopmental disorders in this high-risk population.[Chinese Journal of Contemporary Pediatrics,2024,26(3):297-301]
7.Research progress on neurodevelopmental disorders associated with congenital heart disease
Yu-Mei LIU ; Tian-Yu LIU ; Shao-Han NONG ; Xiao-Guang ZHOU
Chinese Journal of Contemporary Pediatrics 2024;26(11):1231-1237
The incidence and disability rate of neurodevelopmental disorders in children are high,making it a significant public health issue affecting children's health globally. Neurodevelopmental disorders are particularly common in children with congenital heart disease (CHD),with clinical characteristics varying by type of CHD,surgical approach,age stage,and the presence of different complications or comorbidities. In recent years,based on the intervention model of "early diagnosis and early treatment," foreign studies have begun to explore new techniques for preventive early intervention in high-risk children with neurodevelopmental disorders,achieving promising results. This paper reviews the clinical characteristics of neurodevelopmental disorders associated with CHD,aiming to provide a theoretical basis for implementing new preventive early intervention techniques for children with CHD,thereby further reducing the incidence of neurodevelopmental disorders associated with CHD.