Biomedical subjects

Ching-Shiang Chi

Publications and source records attributed to Ching-Shiang Chi.

At least 37 records · Page 2Linked to original sources

PubMed2004

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.

Aneurysm↗

PubMed2003

Lupus vasculopathy combined with acute renal failure in lupus nephritis.

Several risk factors have been associated with the prognosis of lupus nephritis. However, few studies have focused on renal vascular lesions (such as thrombi due to immune complexes) as a prognostic factor in this disease. Here we present a case of systemic lupus erythematosus (SLE) in a 12-year-old girl who exhibited acute renal failure and severe hypertension on admission. Renal pathology findings included diffuse proliferative glomerulonephritis (class IVb) and lupus vasculopathy (LV) with immune complex deposition within glomerular capillaries and the preglomerular arteriolar lumen. Her clinical condition deteriorated rapidly, even after cyclophosphamide and methylprednisolone pulse therapy. It improved after 5 days of plasmapheresis and remained stable for up to 6 months under regular treatment. We suggest that renal biopsy performed early in SLE patients with renal involvement should be studied carefully for the presence of vascular lesions. Additionally, plasmapheresis can be considered in patients with LV refractory to other modalities of therapy.

Acute Kidney Injury↗

PubMed2003

Pulmonary edema of enterovirus 71 encephalomyelitis is associated with left ventricular failure: implications for treatment.

Epidemics of enterovirus 71 infections caused the rapid death of many children in Malaysia in 1997 and in Taiwan in 1998. Pulmonary edema occurred in most of the fatal cases and was considered to be neurogenic. The role of the heart was rarely investigated before. Between January 1998-January 2001, 34 consecutive patients who were admitted to the intensive care unit due to enterovirus infection were studied prospectively. Patients were divided into two groups: group I with pulmonary edema, and group II without pulmonary edema. Comparisons were made between the two groups based upon demographic, neurological, and cardiovascular manifestations. Group I consisted of 11 patients (5 boys, 6 girls; mean age, 22.8 months), and group II of 23 patients (12 boys, 11 girls; mean age, 28.8 months). There were no significant differences between the two groups in comparing sex, age, body weight, neurological severity, intracranial pressure, cell count, protein and glucose levels in cerebral spinal fluid, and blood pressure. All group I patients had left ventricular dysfunction, and their ejection fractions were significantly lower than those of patients in group II (37 +/- 11% vs. 75 +/- 6%, P < 0.001). Group I heart rates were higher than those of group II (175 +/- 24 vs. 137 +/- 25, P < 0.001). In group I, 9 patients who received conventional treatment died, and the only two survivors received left ventricular assist devices. In conclusion, the pulmonary edema of fulminant enterovirus 71 infection is associated with left ventricular failure. Left ventricular function is the major determinant of outcome. Early recognition of heart failure and aggressive cardiac intervention are life-saving. Pediatr Pulmonol. 2003; 35:263-268.

Child, Preschool↗

PubMed2003

Maple syrup urine disease in the Austronesian aboriginal tribe Paiwan of Taiwan: a novel DBT (E2) gene 4.7 kb founder deletion caused by a nonhomologous recombination between LINE-1 and Alu and the carrier-frequency determination.

Maple syrup urine disease (MSUD) is an autosomal recessive inborn error disorder derived from the accumulation of the branched-chain amino acids (BCAAs) leucine, isoleucine and valine. Either the E1alpha, E1beta or DBT (E2) genes are responsible for this neurometabolic disease. Here, we report the identification and characterization of a novel E2 gene 4.7 kb deletion as a rare nonhomologous recombination of the long interspersed nuclear elements 1 (LINE-1) in intron 10 and the Alu in the 3' UTR of the E2 gene from three classic MSUD patients of the Austronesian aboriginal tribe Paiwan in Taiwan. The E2 gene 4.7 kb deletion accounted for five out of six alleles in the three unrelated Paiwanese MSUD patients, indicating a founder effect. Carrier-frequency study revealed one deleted heterozygote out of 101 normal Paiwanese. As the nine Taiwanese Austronesian aboriginal tribes share a common origin, this E2 4.7 kb deletion may be preserved in some of the other Austronesian aboriginal tribes of Taiwan. This is the first comprehensive genetics study of MSUD in the Austronesian tribal groups as well as in Taiwan.

Alu Elements↗

PubMed2003

Influence of ductal size on the results of transcatheter closure of patent ductus arteriosus with coils.

To assess the influence of ductal size on the results of transcatheter closure of patent ductus arteriosus (PDA) with coils, 154 consecutive patients were studied prospectively. Ductal size was defined as the narrowest diameter of ductus measured on aortography. All patients were divided into 5 groups according to ductal size: < 1 mm, 1-1.9 mm, 2-2.9 mm, 3-3.9 mm, and > or = 4 mm. The occlusion of PDA with coils was performed through a transarterial approach. The results were evaluated by angiography at 10 minutes and by color Doppler echocardiography at 1 day, 2 days, 1 week, 1 month, 3 months, 6 months, and 12 months after the procedure. The immediate occlusion rates for ductal sizes < 1 mm, 1-1.9 mm, 2-2.9 mm, 3-3.9 mm, and > or = 4 mm were 89.7%. 75.4%, 51.4%, 30.8%, and 40%, respectively; whereas the occlusion rates at 12-months follow-up were 100%, 98.5%, 97.3%, 69.2%, and 80%, respectively. There were no significant differences in occlusion rate at 12-months follow-up among the groups with ductal sizes < 3 mm or among the groups with ductal sizes > or = 3 mm. The occlusion rate for ductal size < 3 mm at each follow-up time was significantly higher than that for ductal size > or = 3 mm (10 minutes: 71.8% vs 34.8%. P = 0.001; 12-months: 98.5% vs 73.9%, P < 0.001). The occlusion rate of residual shuntings at 12-months follow-up for ductal size < 3 mm was also significantly higher than that for ductal size > or = 3 mm (94.6% vs 60%, P = 0.007). The results of the present study demonstrate that ductal size < 3 mm had a higher occlusion rate than that for a size > or = 3 mm. PDA with a size > or = 3 mm may need other treatment strategies or other devices to achieve better results.

Cardiac Catheterization↗

PubMed2003

Verapamil sensitive idiopathic ventricular tachycardia in an infant.

Idiopathic ventricular tachycardia is rare, especially in infants. We report here on an 8 month-old female infant who presented with tachycardia with a heart rate of 186 beats/ min. An electrocardiogram showed a right bundle branch block pattern, a QRS duration of 80 msec, a superior QRS axis, atrioventricular dissociation, and occasional fusion and capture beats. Suspected ventricular tachycardia was treated with lidocaine, propranolol and amiodarone, but in vain. The tachycardia was terminated and well controlled with the use of verapamil. According to an electrocardiogram and her clinical response, verapamil-sensitive idiopathic ventricular tachycardia was diagnosed with the arrhythmic origin in the left posterior fascicle.

Amiodarone↗

PubMed2003

Brain abscess caused by Salmonella enterica subspecies houtenae in a patient with chronic granulomatous disease.

A 44-month-old boy with chronic granulomatous disease has been suffering from fever and skin rash for 7 days prior to admission. The blood culture obtained on admission revealed Salmonella enterica subspecies houtenae. He received intravenous ceftriaxone therapy during his hospital stay and oral cefixime after discharge. Unfortunately, the same symptoms recurred 2 weeks after discontinuing cefixime and the culture from the aspirate of a skin nodule yielded the same microorganism again. He received intravenous ceftriaxone therapy after readmission and became afebrile 3 days later. However, focal seizure was noted on the 14th day of hospitalization. Brain magnetic resonance imaging revealed multiple brain abscesses, and electroencephalogram showed epileptiform activity. The intravenous antimicrobial agents were continued for a total of 84 days and interferon-gamma was administered as adjunctive therapy. Finally, he recovered from brain abscesses without any neurologic sequel. It is suggested that an extended course of antimicrobial treatment is necessary for chronic granulomatous disease with pyogenic infection because of the defective intracellular killing ability.

Anti-Bacterial Agents↗

PubMed2003

Wolfram syndrome: phenotype and novel mutation in two Taiwanese siblings.

Wolfram syndrome (WS) is a rare autosomal recessive neurodegenerative disorder. The responsible gene, WFS1, was identified in 1998 and over 66 mutations have been reported since then. We report 2 siblings in a Taiwanese family with WS. They had similar clinical courses, including successive development of diabetes mellitus, optic atrophy, diabetes insipidus, hearing impairment, and urological complications from age 5 to 15 years. Rapid progression of systemic and neurological symptoms was noted in the elder brother. Mutation analysis of the 2 probands revealed compound heterozygotes of 1 novel and 1 previously reported mutation. Their parents and an asymptomatic sibling were carriers of 1 mutation.

Blood Platelet Disorders↗

PubMed2002

Omphalocele and gastroschisis in Taiwan.

UNLABELLED: The purpose of this study was to analyse the clinical differences between omphalocele and gastroschisis in Taiwan, with special reference to associated anomalies and outcomes. A retrospective review was conducted of 115 cases seen between January 1990 and June 2000 at two tertiary medical centres in Taiwan. Data included perinatal events and associated anomalies. Of 115 patients, 65 were classified as having gastroschisis and 50 as having omphalocele. Other anomalies were found in 24 omphalocele cases, compared with 23 gastroschisis cases. The range of anomalies associated with omphalocele varied more widely than in the gastroschisis cohort. Of patients with omphalocele and associated anomalies, six had chromosomal abnormalities compared with none of the patients with gastroschisis. In patients with gastroschisis and additional malformations, 17 had gastrointestinal anomalies, the most common of which was intestinal malrotation. A comparison of perinatal data revealed that infants with gastroschisis were more likely to be small for gestational age. Gastroschisis was associated with a younger overall maternal age than omphalocele and a lower birth weight. There was a male predominance among omphalocele patients, but this did not reach statistical significance. CONCLUSION: Gastroschisis was frequently associated with intestinal anomalies and transient dysfunction, and outcomes were related to postoperative complications. Infants with gastroschisis more frequently required prolonged parenteral nutrition supplement, resulting in longer hospital stay. Omphalocele was often accompanied by chromosomal disorders leading to early neonatal death, so we recommend that amniocentesis should be indicated if omphalocele is suspected on fetal ultrasonography.

Abdominal Wall↗

PubMed2002

Left ventricular dysfunction in children with fulminant enterovirus 71 infection: an evaluation of the clinical course.

We describe 2 children with typical hand, foot, and mouth disease due to enterovirus 71 infection, 1 of whom died. Both cases were complicated by acute fulminant shock syndrome; the patients had remarkable acute left ventricular dysfunction. The clinical experience indicates that the rapid death associated with fulminant enterovirus rhombencephalitis is the result of rapid cardiogenic shock rather than neurogenic pulmonary edema.

Child, Preschool↗

PubMed2002

Isolated neonatal ductus arteriosus aneurysm.

OBJECTIVE: A prospective study was performed to evaluate the incidence, clinical manifestations and outcome of ductus arteriosus aneurysm (DAA) in full-term neonates. BACKGROUND: Ductus arteriosus aneurysm has been considered to be a rare congenital lesion and a potentially fatal abnormality. METHODS: A total of 548 full-term neonates received echocardiographic screening. RESULTS: There were 48 (8.8%) patients (28 boys and 20 girls) with DAA detected by echocardiography. The maximal diameter of the DAA ranged from 6.5 to 11.2 mm (8.2 +/- 1.2 mm). All cases were asymptomatic. There were no significant differences in gender, gestational age, maternal age or Apgar score between the newborns with or without DAA. Newborns with DAA had a higher birth body weight, higher incidence of maternal gestational diabetes mellitus and more mothers with blood group A, compared with newborns without DAA (p < 0.05). Follow-up echocardiograms showed spontaneous closure of the ductus arteriosus in all patients except those without DAA. The DAA became progressively smaller after ductal closure in 33 patients (70.2%) and completely disappeared by 7 to 35 days of life. The other 14 patients (29.8%) with DAA had echocardiographic evidence of progressive formation of thrombi between the third and tenth day of life. The DAA and thrombi spontaneously disappeared in all patients by one month after birth. CONCLUSIONS: There is a higher incidence of DAA with a good outcome in our series compared with previous reports. We speculate that the presence of DAA may be a normal variant of the ductal bump and part of a normal process of spontaneous ductal closure in full-term neonates.

Aneurysm↗

PubMed2002

Discordance between dipyridamole stress technetium-99m tetrofosmin single photon emission computed tomography and coronary angiography in patients with Kawasaki disease.

Kawasaki disease (KD) is an acute vasculitis syndrome of unknown etiology that mainly affects the coronary arteries. The purpose of this study was to assess the agreement between dipyridamole stress technetium-99m tetrofosmin (Tc-TF) myocardial perfusion single photon emission computed tomography (SPECT) and coronary angiography in these patients. Twenty-nine children with KD were included in this study. All of the 29 children also received dipyridamole stress Tc-TF myocardial perfusion SPECT within 1 month of their coronary angiographic studies. The results showed that (1) 89.7% of children had negative coronary angiographic findings without significant coronary stenoses, and 10.3% of children had positive coronary angiographic findings with significant coronary stenosis; (2) 44.8% of children had negative Tc-TF myocardial perfusion SPECT findings without abnormal myocardial perfusion, and 55.2% of children had positive Tc-TF myocardial perfusion SPECT findings with abnormal myocardial perfusion; (3) 44.8% of children had both normal coronary angiographic and Tc-TF myocardial perfusion SPECT findings, and 10.3% of children had both abnormal coronary angiographic and Tc-TF myocardial perfusion SPECT findings; and (4) There was no significant agreement between coronary angiographic and Tc-TF myocardial perfusion SPECT findings. We concluded that poor agreement exists between coronary angiographic and Tc-TF myocardial perfusion SPECT findings with coronary stenoses and abnormal myocardial perfusion in children with KD.

Adolescent↗

PubMed2002

Menkes gene study in the Chinese population.

We sought to determine the nature of the molecular defect causing Menkes' syndrome in the Chinese population. The DNA of four patients with typical Menkes manifestation was sequenced. Two pathologic genetic defects were identified; one of them is a nonsense mutation, whereas the other is a frameshift mutation. Both of these mutations are de novo.

Adenosine Triphosphatases↗

PubMed2002

Clinical outcome of invasive pneumococcal infection in children: a 10-year retrospective analysis.

A retrospective study was conducted on 72 children admitted to a medical center in Taiwan due to invasive pneumococcal infections diagnosed between January 1990 and April 2000. Of these patients, 28 had meningitis and 44 had other invasive diseases. Forty-one (56.9%) strains of Streptococcus pneumoniae showed reduced susceptibility to penicillin by the oxacillin disc diffusion method. The total mortality was 20.8%, 32.1% for meningitis, and 13.6% for other invasive diseases. Ten (52.6%) of the patients survived from meningitis had long-term sequelae. Statistical analysis showed that initial presentation of coma, shock, respiratory distress requiring mechanical ventilation, and leukopenia (leukocyte <4,000 /mm3) were associated with mortality of invasive pneumococcal infections. Low cerebrospinal fluid leukocyte count (<50 /mm3) and high cerebrospinal fluid protein level (> or = 660 mg/dL) were also associated with mortality of meningitis. The presence of underlying diseases and high alanine aminotransferase level (> or = 100 U/L) were associated with fatal non-meningitic invasive diseases. Patients with shock and high alanine aminotransferase level but without high C-reactive protein level (> or = 20 mg/dL) were associated with rapidly fatal outcome. The outcome of invasive pneumococcal diseases was not associated with penicillin susceptibility.

Adolescent↗

PubMed2002

Clinical experience of managing empyema thoracic in children.

Data of 54 children with a diagnosis of thoracic empyema at a medical center in central Taiwan from January 1991 through April 2001 were analyzed. Their mean age was 4.4 years and the mean hospital stay was 13 days. Streptococcus pneumoniae was the most common pathogen, followed by Staphylococcus aureus and Haemophilus influenzae. These patients were divided into 2 groups according to the treatment method. Twenty-two patients were treated successfully with antibiotics and tube thoracostomy, whereas the other 32 children required further pleural decortication with antibiotic treatment. In patients with empyema, decortication allowed for more rapid defervescence than did closed tube thoracostomy (1.94 vs 5.04 days; p<0.001) and there were no complications in the group that underwent decortication treatment (p<0.03). In conclusion, the decortication of loculated empyema thoracis in children is a safe and effective management procedure.

Adolescent↗

PubMedDate not supplied

Discordance between dipyridamole stress Tc-99m sestamibi SPECT and coronary angiography in patients with Kawasaki disease.

BACKGROUND: Kawasaki disease is an acute vasculitis syndrome of unknown etiology that mainly affects the coronary arteries. The purpose of this study was to assess the agreement between dipyridamole stress technetium 99m sestamibi single photon emission computed tomography (SPECT) and coronary angiography in these patients. METHODS AND RESULTS: Forty-one consecutive patients (29 boys and 12 girls) who underwent coronary angiography were studied prospectively. Their ages at onset of the disease ranged from 2 months to 4.8 years (mean +/- SD, 1.9 +/- 1.3 years). Their ages at the time of the study ranged from 8 months to 15.3 years (6.2 +/- 4.4 years). The duration between symptom onset and the study ranged from 2 months to 12 years (4.3 +/- 4.0 years). All patients underwent dipyridamole stress Tc-99m sestamibi SPECT within 1 month of their angiographic studies. They were divided into 3 groups according to coronary angiography findings. Group A consisted of 2 patients (1 boy and 1 girl, aged 10.3 and 1.9 years, respectively) with coronary stenoses who also had aneurysms. Group B consisted of 10 patients (8 boys and 2 girls, aged 0.7-15.3 years [mean, 3.8 years]) with coronary aneurysms. Group C consisted of 29 patients (20 boys and 9 girls, aged 1.2-13.8 years [mean, 7 years]) with normal coronary angiograms. Two patients in group A (100%), 3 of 10 patients in group B (30%), and 19 of 29 patients in group C (65.5%) had myocardial perfusion defects. There was poor agreement between Tc-99m sestamibi SPECT and coronary angiography for detecting coronary stenoses (kappa = 0.07; P =.222) and aneurysms (kappa = -0.184; P =.158). CONCLUSION: Significant discordance exists between Tc-99m sestamibi SPECT and coronary angiography in patients with Kawasaki disease.

Adolescent↗

PubMedDate not supplied

Spondyloepiphyseal dysplasia tarda: report of one case.

We report the clinical assessment and mutational analysis of one boy with spondyloepiphyseal dysplasia tarda (SEDT). His short stature manifested between age 5 and 10. As his growth slowed, his height fell from the mean for his age at 5 to the 2nd percentile at age 10.5. His bone age was retarded at 8 years, thus his predicted adult height was 164.9 cm, not far from his target height of 170cm. However, upon physical examination, he presented markedly short trunk. We suspected SEDT and X-ray finding of his spine supported this diagnosis. We performed DNA analysis on the SEDL gene and detected a 5-bp deletion in exon 5 which has been previously reported by Mumm et al. and Gedeon et al. in cases of SEDT.

Carrier Proteins↗

PubMedDate not supplied

Management of acute decompensation of neonatal maple syrup urine disease with continuous arteriovenous haemofiltration: report of one case.

A 22-day-old female infant was referred to our hospital due to unusual urine odor suspecting inborn error of metabolism. Physical examination revealed a stuporous and hypotonic infant with poor reflexes. Intravenous thiamine and high glucose along with appropriate protein were given under the suspicion of maple syrup urine disease (MSUD), which was confirmed by blood and cerebrospinal fluid amino acid assays, and urinary organic acid assay. Progressive neurological deterioration was observed despite the non-invasive treatment. So, we performed pump assisted continuous arteriovenous haemofiltration (CAVHF). Dramatic improvement in neurological function was observed hours after initiation of CAVHF, along with decrease in the level of isoleucine, leucine and valine. In our experience, CAVHF is a well-tolerated procedure for managing the acute phase of neonatal MSUD. Further study on indications, duration of treatment, and preventing complications is needed.

Acute Disease↗