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Biomedical subjects

Ching-Shiang Chi

Publications and source records attributed to Ching-Shiang Chi.

At least 19 recordsLinked to original sources

Acute immune thrombocytopenic purpura in infants: associated factors, clinical features, treatment and long-term outcome.

The natural course of acute immune thrombocytopenic purpura (ITP) in infants is poorly described in the literature. A retrospective study of 17 consecutive patients <1 yr of age admitted and treated for acute ITP between 1996 and 2005 was conducted. We investigated their demographics, vaccination history, clinical features, laboratory examinations, response to treatment and long-term outcome. There were 11 male and six female infants. Their ages ranged from 24 d to 12 months with a median of 3 months. All infants presented with petechiae and/or ecchymoses. Fourteen cases had platelet counts below 20 x 10(9)/L at the time of admission. They all had good response to a single course of treatment (14/17) or multiple courses of treatment (3/17). None had progressed into chronic ITP. Seven infants had a causal relationship with immunization, five associated with hepatitis B, one diphtheria-pertussis-tetanus, one diphtheria-tetanus-acellular pertussis-inactivated poliovirus vaccine-conjugated Haemophilus influenza vaccines. These seven infants responded to treatment within 3-9 d after therapy with intravenous immunoglobulin, high-dose methylprednisolone or oral steroids. Re-boosters with vaccines revealed no recurrence of the disease in all of these seven patients. The study suggests that further immunization is not contraindicated in infants experiencing acute ITP associated with vaccines.

Female↗

Norepinephrine induces apoptosis in neonatal rat endothelial cells via a ROS-dependent JNK activation pathway.

Our previous study demonstrated that norepinephrine (NE) induces endothelial apoptosis mainly through down-regulation of Bcl-2 protein and activation of the beta-adrenergic and caspase-2 pathways. However, whether reactive oxygen species (ROS) and mitogen-activated protein kinases (MAPKs) are involved in this signal transduction remains unknown. Endothelial cells cultured from neonatal rat heart were treated with 100 microM NE. Proteins of MAPKs and Bcl-2 family were assayed by Western blotting. Apoptosis was determined by terminal deoxynucleotidyl transferase-mediated nick end-labeling assay. ROS was analyzed with flow cytometry. Caspase activity was measured using specific fluorogenic substrates. Treatment with NE increased intracellular ROS level and extracellular signal-regulated kinase (ERK), c-Jun N-terminal kinase (JNK), and p38 phosphorylation. Whereas the phosphorylated form of Akt was decreased. The NE-induced apoptosis was abrogated by SP600125 (a specific inhibitor of JNK). Antioxidants such as vitamin C and N-acetyl cysteine inhibited NE-induced ROS production, JNK phosphorylation, caspase activation and apoptosis. Exogenously added superoxide dismutase or catalase markedly diminished NE-induced ROS production and cell death. In conclusions, our study is the first report documenting that NE induces apoptosis in neonatal rat endothelial cells via a ROS-dependent JNK activation pathway. Antioxidants may be useful in the prevention and management of NE-mediated endothelial apoptosis during heart failure.

Acetylcysteine↗

Corneal clouding: An infrequent ophthalmic manifestation of mitochondrial disease.

Corneal clouding is uncommon in infants and children but when present in this age group, it is often associated with mucopolysaccharidoses or Fabry disease. This report describes the case of an 11-year-old male who demonstrated poor weight gain, short stature, segmental myoclonus, and learning problems from 5 years of age followed by general weakness and extremely poor balance. Corneal clouding was evident as a result of a blurred vision complaint at 9 years of age. Both urine metabolic screening for mucopolysaccharidoses and analysis of lysosomal enzymes displayed negative findings. Clinical conditions worsened, including ptosis, progressive weakness, and positive Gowers' sign. Oral glucose lactate stimulation test was positive, therefore a muscle biopsy was performed at 11 years of age. Light microscopy of muscle biopsy disclosed abundant ragged red fibers; electron microscopy revealed abnormal mitochondria in terms of tubular cristae, concentrated cristae, stacking cristae, and round granular patterns of inclusion bodies in the matrix. Thus mitochondrial disease was diagnosed. We conclude that mitochondrial disease should be added to the list of differential diagnosis of corneal clouding in children, especially in cases with normal urine metabolic screening for mucopolysaccharidoses or when assays of lysosomal enzymes appear normal.

Child↗

High-dose norepinephrine induces disruption of myocardial extracellular matrix and left ventricular dilatation and dysfunction in a novel feline model.

BACKGROUND: Intravenous norepinephrine (NE) at a dose of 1-6 microg/kg/minute can induce increased extracellular matrix (ECM) and hypertrophic cardiomyopathy. This study aimed to investigate the effects of a higher dose of NE on cardiac remodeling. METHODS: After intraperitoneal urethane-chloralose anesthesia, 7 cats (3.03 +/- 0.58 kg) received intravenous infusion of NE 30 microg/kg/minute for 3 hours. Aortic blood pressure and heart rate (HR) were measured by polygraphy at 0, 5, 15, 30, 60, 90, 120, and 180 minutes. Left ventricular size and ejection fraction (EF) were measured by M-mode echocardiography before and after NE administration. Histopathology was performed by hematoxylin-eosin, silver impregnation, and Sirius red staining. Activity of matrix metalloproteinases (MMP) in the left ventricle was measured by zymography. RESULTS: Mean blood pressure (mmHg) increased from 139 +/- 20 to 198 +/- 19, 187 +/- 23, and 166 +/- 16 at 15, 30, and 60 minutes, respectively, during NE infusion. HR (beats/minute) decreased from 214 +/- 10 to 158 +/- 28 at 15 minutes and then recovered gradually. The left ventricles showed significant dilatation (end-diastolic diameter: from 1.20 +/- 0.18 to 1.58 +/- 0.23cm, p=0.003; end-systolic diameter: from 0.62 +/- 0.23 to 1.35 +/- 0.29cm, p=0.002) and hypokinesia (EF: from 86.2 +/- 5.2 to 33.1 +/- 16.5%, p < 0.001). Histopathology revealed that left ventricular myocytes were elongated, wavy, and fragmented, while collagen fibers were overstretched, straightened, and disrupted. MMP-9 activity was significantly elevated (p = 0.003 vs. control), while MMP-2 activity was unchanged. CONCLUSION: High-dose NE increases MMP-9 activity and causes ECM disruption, left ventricular dilatation and dysfunction.

Animals↗

Radiographic, clinical, and prognostic features of complicated and uncomplicated community-acquired lobar pneumonia in children.

BACKGROUND AND PURPOSE: The incidence of community-acquired lobar pneumonia in conjunction with either necrosis or empyema in children has rapidly increased in recent years. This study aimed to evaluate the radiographic, clinical, and predicted factors of complicated and uncomplicated lobar pneumonia in children. METHODS: This retrospective study included 131 patients younger than 18 years of age with community-acquired lobar pneumonia treated between January 2002 and March 2005. Multiple logistic regression analysis was performed to demonstrate the risk factors of complicated lobar pneumonia. RESULTS: The proportion of children with lobar pneumonia in children increased dramatically from 7% in 2002 to 19% in 2004. Analysis revealed the presence of elevated C-reactive protein level (>12 mg/dL) [odds ratio (OR), 3.51; 95% confidence interval (CI), 1.61-7.66], persistent fever for more than 1 week before admission (OR, 1.14; 95% CI, 1.04-1.26), and multilobar (> or =2 lobes) confluent lung opacity on chest radiographs (OR, 2.83; 95% CI, 1.27-6.33) were independent predictors of the occurrence of complicated lobar pneumonia. A progressive increase in the number of penicillin-non-susceptible Streptococcus pneumoniae isolates was found during the study period. Prolonged fever was a common clinical feature of hospitalized children with lobar pneumonia. Failure of consolidative pneumonia to respond to appropriate antibiotic treatment within 4.4 days was associated with the development of necrosis or empyema. CONCLUSIONS: Complicated and uncomplicated lobar pneumonia are difficult to distinguish based on clinical symptoms at the time of admission. The presence of the above risk factors can help in the early diagnosis of complicated lobar pneumonia.

C-Reactive Protein↗

Initial clinical experience of real-time three-dimensional echocardiography in neonates with isolated congenital ductus arteriosus aneurysm.

BACKGROUND: Congenital ductus arteriosus aneurysm (DAA) was considered rare but potentially fatal abnormality, often followed by surgical intervention after careful evaluation. This prospective study used real-time three-dimensional echocardiography (RT3DE) to assist in evaluation of neonatal DAA. METHODS: A total of 1390 full-term neonates were enrolled in this study between 2002 and 2003. They received two-dimensional echocardiographic (2DE) screening and periodic follow-up. RT3DE was performed selectively for newborns with DAA. RESULTS: DAA were detected in 116 (8.34%) newborns using 2DE. Maximum diameter of the DAAs ranged from 6.8 to 14.0 mm (8.2+/-1.1 mm). None of the cases were symptomatic or had complications related to DAA. There were no significant differences in sex and gestational age between the newborns with and without DAA. Neonates with DAA had a higher birth body weight and a higher incidence of large-for-gestational-age (P<0.05). RT3DE provided instant, consistent and reliable 3D images of DAA and its related structures and allowed for more rapid examination times and reduction of baby wait times. CONCLUSIONS: Congenital DAA is as common as has been previously reported. RT3DE is useful in assisting evaluation of DAA. Preferred images of DAA were typically visualized in the high parasternal short-axis view before the third day of life. Routine use of RT3DE is suggested to enhance assessment of neonates with DAA detected by 2DE.

Ductus Arteriosus↗

A rare vascular ring caused by an aberrant right subclavian artery concomitant with the common carotid trunk: a report of two cases.

In this report, we describe two rare cases with aortic branching anomalies. One was a 7-year-old girl who had an incomplete vascular ring caused by the presence of left aortic arch and aberrant, retroesophageal right subclavian artery associated with the common carotid trunk. The other was a 6-year-old boy who had a right aortic arch and mirror image branches of the former case. Both were asymptomatic and discovered incidentally. Three-dimensional surface rendering magnetic resonance angiography was used to confirm the diagnosis. Surgical intervention was not performed in either case because of lack of vascular ring-related symptoms. The incidence, clinical symptoms, pathogenesis and management of this rare vascular ring are discussed below.

Aortic Arch Syndromes↗

Sympathetic activity and myocardial damage after stimulation of dorsal medulla and vagotomy in a novel animal model.

Many kinds of brain lesions cause sympathetic hyperexcitation and myocardial damage. A novel animal model was developed for the correlation of sympathetic excitation with ventricular dysfunction and myocardial damage. Six cats (3.23+/-0.26 kg) under intraperitoneal urethane-chloralose anesthesia were artificially ventilated and bilaterally vagotomized. They underwent an electrical stimulation of unilateral dorsal medulla for 180 min (monopolar square-wave pulses, 10 Hz, 10 V, 0.5 ms). Mean blood pressure, heart rate plasma concentration of norepinephrine and left ventricular size and ejection fraction were measured at 0, 5, 15, 30, 60, 90, 120 and 180 min. Mean blood pressure (mm Hg), heart rate (beats/min) and norepinephrine (pg/ml) increased abruptly from 128+/-15, 203+/-22 and 353+/-123 to 234+/-26, 240+/-13 and 4727+/-2159 at 5 min after electrical stimulation (all p<0.01). The left ventricles showed significant dilatation (end-diastolic diameter: from 1.35+/-0.13 to 1.84+/-0.21 cm, p=0.006; end-systolic diameter: from 0.65+/-0.20 to 1.54+/-0.24 cm, p=0.002) and hypokinesia (ejection fraction: from 88.9+/-6.4% to 37.3+/-8.7%, p<0.001). Cardiac pathology revealed myocardial hemorrhage, cardiomyocyte apoptosis and coagulative myocytolysis (contraction band necrosis), characterized by sarcoplasmic coagulation, granulation and disruption. In conclusions, the present experiment develops a novel animal model in which stimulation of the pressor area in the dorsal medulla in vagotomized cats produces sympathetic hyperexcitation accompanied with myocardial dysfunction and damage. This model may be applicable for studying protective effect of drugs on myocardial dysfunction and damage caused by sympathetic hyperexcitation occurring in brain diseases.

Animals↗

Congenital chylothorax associated with isolated congenital hypoplastic superior caval vein: a case report.

UNLABELLED: Congenital absence, or hypoplasia, of the superior vena cava (SVC) with situs solitus is quite uncommon. Most cases have been found incidentally and concomitant with left persistent SVC or other cardiac disorders. Congenital chylothorax may be associated with cardiac anomalies, various syndromes, thoracic ductal or venous thrombosis, birth trauma or local compression of the thoracic duct, but it is rarely caused directly by congenital SVC anomalies. CONCLUSION: We report a rare case of congenital chylothorax associated with isolated congenital hypoplastic SVC and underdeveloped collateral circulation.

Abnormalities, Multiple↗

Norepinephrine induces apoptosis in neonatal rat cardiomyocytes through a reactive oxygen species-TNF alpha-caspase signaling pathway.

OBJECTIVE: Norepinephrine (NE)-induced apoptosis in cardiomyocytes is an important cause of heart failure. Previous studies revealed that reactive oxygen species (ROS) are involved in apoptosis. Tumor necrosis factor-alpha (TNF), a well-known mediator that stimulates apoptosis, is not only produced by macrophages but also by cardiomyocytes. Until now, the role of TNF and its relationship to ROS in NE-induced apoptosis of cardiomyocytes have never been investigated. METHODS: Neonatal rat cardiomyocytes were treated with various concentrations of NE. Apoptosis of cardiomyocytes was determined using the TUNEL assay. The level of secreted TNF was measured by ELISA and TNF mRNA expression was determined by semiquantitative reverse transcriptional polymerase chain reaction. Caspase activity was measured by a fluorogenic protease assay kit. Anti-TNF antibodies, caspase inhibitors and antioxidants (N-acetyl-L-cysteine or vitamin C) were added to determine if they could inhibit the apoptotic effect of NE. RESULTS: NE induced apoptosis of cardiomyocytes in a dose- and time-dependent manner. NE up-regulated TNF mRNA expression and increased TNF secretion and caspase-2,-3,-6, and -9 activities. A neutralizing anti-TNF antibody and caspase-2 and -3 inhibitors significantly attenuated NE-induced apoptosis. Antioxidants completely abrogated NE-induced TNF secretion, caspase activation, and apoptotic death. CONCLUSION: NE induced apoptosis in neonatal rat cardiomyocytes through a ROS-TNF-caspase signaling pathway.

Animals↗

Norepinephrine induces apoptosis in neonatal rat endothelial cells via down-regulation of Bcl-2 and activation of beta-adrenergic and caspase-2 pathways.

OBJECTIVES: Heart failure is associated with increased plasma norepinephrine (NE) and endothelial apoptosis. Recent reports have suggested that endothelial dysfunction is an important target for future therapies of heart failure. However, whether NE can induce endothelial apoptosis and its mechanism remains unknown. METHODS: Endothelial cells from neonatal rat heart were treated with various concentrations of NE for different durations. Apoptosis was assessed by terminal deoxynucleotidyl transferase-mediated nick end-labeling (TUNEL) and DNA fragmentation assays. Caspase activity was measured using specific fluorogenic substrates. Proteins of Bcl-2 family and cytochrome c were assayed by Western blotting. RESULTS: NE induced endothelial apoptosis in a dose- and time-dependent manner. After treatment for 48 h, increasing NE concentration from 5, 10, 50, 100 to 200 microM resulted in 6+/-3%, 14+/-5%, 43+/-4%, 66+/-5%, and 89+/-6% apoptotic cells, respectively. The apoptosis was accompanied by down-regulation of Bcl-2 protein synthesis but not by cytosolic cytochrome c translocation. Caspase-2, -3, -6 and -9 were activated during apoptosis and caspase-2 inhibitor (Z-VDVAD-FMK) and caspase-3 inhibitor (Z-DEVD-FMK) significantly attenuated the apoptosis. Overexpression of Bcl-2 inhibited caspase activity and decreased the apoptosis. Moreover, the NE-mediated apoptotic effect was attenuated by beta- (beta2>beta>beta1) adrenergic antagonists (ICI-118,551>propranolol>atenolol) but was not affected by alpha1- or alpha2-adrenergic antagonists (prazosin or yohimbine). CONCLUSION: Our study is the first report documenting that NE induces apoptosis in neonatal rat endothelial cells mainly through down-regulation of Bcl-2 protein and activation of the beta-adrenergic (beta2>beta1) and caspase-2 pathways. beta-Adrenergic antagonists and caspases inhibitors may be useful in the prevention and management of NE-mediated endothelial apoptosis during heart failure.

Adrenergic beta-Antagonists↗

Paralytic ileus in MELAS with phenotypic features of MNGIE.

This report describes a child having the syndrome of overlapping phenotypic features of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and mitochondrial neurogastrointestinal encephalopathy syndrome (MNGIE). Mitochondrial DNA analysis revealed a point mutation at position A3243G, whereas activity of thymidine phosphorylase and its corresponding gene analysis were normal. The most striking feature of this case was paralysis of one segment of the terminal ileum observed on laparotomy. The electron microscopic findings of the resected ileum and colon by limited right hemicolectomy disclosed accumulation of numerous enlarged mitochondria with ill-defined cristae which were similar to mitochondria reported in three previous MELAS cases and one MNGIE case with intestinal dysmotility. We emphasize that the MELAS and MNGIE phenotypes overlapped in this case and that the mechanism of acute ileus in MELAS was associated with functional paralysis of the intestine.

Acute Disease↗

Prediction of ductus arteriosus closure by neonatal screening echocardiography.

AIMS: We reported the incidence, clinical manifestations and outcome of patent ductus arteriosus (PDA) in full-term infants by screening echocardiography. METHODS AND RESULTS: Total 1230 infants received echocardiographic screening and periodic follow-up. On the third day of life, there were 109 infants with isolated ductus arteriosus aneurysm (DAA), those with persistent patency of the ductus arteriosus (DA) in 26. The DA closed within 48 h in 828 infants, and persisted beyond 48 h in 180. The incidence of PDA in full-term infants was 0.91, 0.83 and 0.66% at 1, 4 and 6 months follow-up, and 0.82, 0.73 and 0.64% if infants with DAA were excluded. Infants with PDA had lower birth body weight (BBW) than those with closed DA (p = 0.02). The DA width on the third day of life ranged from 0.23 to 0.43 cm (0.32+/-0.08 cm) and 0.08 to 0.44 cm (0.16+/-0.06 cm) in infants with or without PDA, respectively, which was significantly different (p < 0.05). The sensitivity, specificity, predictive positive and negative values were 87.5, 92.5, 36.8 and 99% respectively for the cuff-off point at 0.25 cm of DA width. CONCLUSIONS: Risk factors in infants with PDA included lower BBW and larger measured DA width at the third day of life. We predicted the arterial duct would close if DA width <0.25 cm, and suggested follow up earlier if >0.25 cm on the third day of life.

Cardiac Surgical Procedures↗

Levamisole in steroid-sensitive nephrotic syndrome children with frequent relapses and/or steroid dependency: comparison of daily and every-other-day usage.

Steroid dependency (SD) and frequent relapses (FR) are common with steroid-sensitive nephrotic syndrome (SSNS). We assessed the effectiveness of daily levamisole in 36 children with SSNS with FR and/or SD. Twenty patients (group 1) were given levamisole 2-3 mg/kg q.o.d. for 4-24 months. Sixteen (group 2) had relapses within 3 months: 5 received levamisole q.d. for 3-18 months, and 11 q.d. for 6 months and then q.o.d. for 4-18 months. Follow-up was 4-36 (mean 20.4 +/- 9.2) months. After therapy, relapses (4.82 +/- 3.15 vs. 2.01 +/- 2.5 in group 1; 5.97 +/- 3.38 vs. 1.34 +/- 2.1 in group 2; p < 0.05) and prednisolone doses (0.57 +/- 0.37 vs. 0.15 +/- 0.33 mg/kg/day in group 1; 0.61 +/- 0.42 vs. 0.19 +/- 0.35 mg/kg/day in group 2; p < 0.05) decreased. Relapse frequency, prednisolone dose, response percentage, and survival curves for remission did not differ between groups. Group 1 had five episodes of leukopenia, and group 2 had four. White blood cell counts normalized after levamisole was discontinued. Serum blood urea nitrogen/creatinine and alanine aminotransferase/aspartate aminotransferase levels were normal. Levamisole is effective in maintaining remission in children with SSNS and FR and/or SD. Daily levamisole can be considered when responses to q.o.d. usage are unsatisfactory.

Adolescent↗

Denys-Drash syndrome.

We report a case of Denys-Drash syndrome, a disorder characterized by male pseudohermaphroditism, congenital nephrotic syndrome, and early renal failure. The patient received dialysis therapy from 15 days of age until his death at the age of 6 months. DNA analysis was performed on the WT1 gene, and a missense point mutation was detected in exon 8 (R366H). After prenatal confirmation of normal WT1 gene in the family's next child, they had a healthy baby 14 months after the patient's death.

Denys-Drash Syndrome↗