Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 19 recordsLinked to original sources

PubMed2004

Cardiac complications of enterovirus rhombencephalitis.

BACKGROUND: Epidemics of enterovirus 71 infection have caused the death of many children throughout the world. Rhombencephalitis, brain stem encephalitis, and heart failure were present in all of the fatal cases. However, no evidence of myocarditis was noted in the heart specimens, and the mechanism of heart failure remains unknown. AIMS: To characterise the presentation of cardiac complications in children with enterovirus rhombencephalitis and discuss its pathogenesis. METHODS: Ninety one consecutive patients with enterovirus rhombencephalitis underwent echocardiography. Of these, 17 patients (nine male, eight female; median age 14 months, range 4-57 months) with left ventricular dysfunction were studied. RESULTS: Tachycardia was noted in all patients and systemic hypertension in 12. Muscle-brain fraction of creatine kinase was >5% in 14 patients. Plasma norepinephrine and epinephrine levels were significantly raised in the three patients in whom these were analysed. Electrocardiographic abnormalities were noted in eight patients. Pulmonary oedema was complicated in 15 patients. The initial ejection fraction of the left ventricle was 22-58% (mean 37%, SD 11%). All patients deteriorated to hypotensive shock within 12 hours and 13 died. Heart specimens from seven patients showed no evidence of myocarditis, but significant coagulative myocytolysis, myofibrillar degeneration, and cardiomyocyte apoptosis were observed. CONCLUSIONS: Acute heart failure was noted in 19% of patients with enterovirus rhombencephalitis, which had a fatality rate of 77%. It was not caused by myocarditis but possibly by neurogenic cardiac damage.

Blood Pressure↗

PubMed2003

Poor agreement between dipyridamole-stress technetium-99m-tetrofosmin myocardial perfusion single photon emission computed tomography and two-dimensional echocardiography in Kawasaki disease.

To establish a noninvasive diagnostic method for early assessment of cardiac involvement in Kawasaki disease (KD), 28 children with KD were included in this study. Two-dimensional echocardiography (2D-Echo) to detect the aneurysms of coronary arteries (CA), as well as rest and dipyridamole-stress technetium-99m tetrofosmin (Tc-TF) myocardial perfusion single photon emission computed tomography (SPECT) to detect abnormal myocardial perfusion were performed in all of the 28 children with KD and to compare each other. The results showed that (1). 42.9% of cases had no aneurysm and 57.1% had significant aneurysms detected by 2D-Echo; (2). 42.9% of cases had normal perfusion and 57.1% of cases had abnormal myocardial perfusion assessed by Tc-TF myocardial perfusion SPECT; (3). 25.0% of cases showed both normal 2D-Echo and Tc-TF myocardial perfusion SPECT findings and 39.3% of cases showed both abnormal 2D-Echo and Tc-TF myocardial perfusion SPECT findings; and (4). there was poor agreement between 2D-Echo and Tc-TF myocardial perfusion SPECT findings (P>0.05). We concluded that poor agreement exists between aneurysms and abnormal myocardial perfusion detected by 2D-Echo and Tc-TF myocardial perfusion SPECT findings in patients with KD.

Adolescent↗

PubMed2003

Mycobacterium avium complex-associated hemophagocytic syndrome in systemic lupus erythematosus patient: report of one case.

Hemophagocytic syndrome (HPS) in systemic lupus erythematosus(SLE) patients has not commonly been reported. In this case study, we report the first case of Mycobacterium avium complex (MAC)-associated hemophagocytic syndrome in a patient with systemic lupus erythematosus (SLE). This SLE patient, a 15-year-old girl, had been on a high dose of prednisolone (> 0.5mg/kg/day) for more than 3 years. She presented with a spiking fever, hepatosplenomegaly, pancytopenia, hyperferritinemia and adult respiratory distress syndrome. Bone marrow examination revealed hemophagocytosis as well as non-caseating granulomatosis. There was no indication of SLE fare-up. She responded poorly to initial treatment with methyl-prednisolone, intravenous immumoglobulin, etoposide, and drugs for Mycobacterium tuberculosis including rifampin, ethambutol, isoniazid and pyramide. However, gastric lavage culture revealed MAC. Following treatment with clarithromycin, ciprofloxacin and amikacin, her condition gradually improved and she was discharged 3 months after admission. In SLE patients with pancytopenia and hyperferritinemia, MAC-associated HPS should be considered in the differential diagnosis.

Adolescent↗

PubMed2002

A novel mutation in neonatal isolated sulphite oxidase deficiency.

Isolated sulphite oxidase deficiency (ISOD) is a very rare hereditary metabolic disorder. Imaging findings of the neonatal form of ISOD, including multicystic leukoencephalopathy with brain atrophy, resemble those of severe ischemic changes of the brain. We report the case of a ten-month-old boy who exhibited neonatal seizures on the 24th day after birth. Excessive quantities of sulphite and S-sulphocysteine in the urine and normal blood uric acid were noted. These findings were consistent with those of ISOD. Point mutations were found in two alleles of the sulphite oxidase (SUOX) gene. One of the mutations was a 1029 C > G mutation, which resulted in an amino acid substitution of tyrosine to a stop code (Y343 X); and the other was a 479 G > A mutation, which resulted in an amino acid substitution of arginine to glutamine (R160 Q). Y343 X is a novel SUOX gene mutation. A review of the literature, including data from this report, showed that 3 of 6 cases had typical imaging findings characterized by initial cerebral edema followed by dramatic multicystic leukoencephalopathy. We emphasize that neonatal ISOD should be included in the differential diagnosis of neonates with unexplained hypoxic-ischemic changes on neuroimaging studies.

Deficiency Diseases↗

PubMed2001

Intra-abdominal actinomycosis with hepatic pseudotumor and xanthogranulomatous pyelonephritis in a 6-y-old boy.

We report the case of a 6-y-old boy with actinomycosis, presenting as xanthogranulomatous pyelonephritis (XGP), hepatic pseudotumor and abdominal abscess. Symptoms included intermittent fever, abdominal pain and significant weight loss. Hepatic and renal tumor masses were suspected on sonography and computerized tomography. XGP and actinomycosis were proven by pathology. The patient recovered well with antibiotic alone.

Abdominal Abscess↗

PubMed2001

Clinical spectrum of Kawasaki disease in infants.

BACKGROUND: Kawasaki disease is a common acquired heart disease in children. Only a few reports have been published concerning Kawasaki disease in infants. This study was performed to assess the clinical spectrum of Kawasaki disease in infants. METHODS: Between January 1989 and December 1998, a total of 48 consecutive Kawasaki patients less than one year of age were enrolled and studied retrospectively. Coronary artery dilation was defined as the internal diameter of a coronary artery larger than 3 mm. All cases received 2 gm/Kg of intravenous immunoglobulin. We divided the patients into two groups; group I; coronary artery dilation (+) and group II; coronary artery dilation (-), and compared the clinical and laboratory data. RESULTS: Of 273 patients with Kawasaki disease, 48 (17.5%) were less than one year of age. Among these patients (< 1 year old), the median age was 7.8 +/- 2.8 months (range 2 months to 12 months), and the male to female ratio was 1.52:1. The incidence of atypical Kawasaki disease was 31.2% (compared with an incidence of atypical Kawasaki disease among patient more than one year of age of 7.5%; p < 0.001), and that of coronary artery dilation was 35.4%. Clinical manifestations included fever 100%, extremity change 91.6%, skin rash 89.6%, conjunctivitis 89.6%, oral mucosa change 89.6%, and cervical lymphadenopathy 0%. Laboratory data revealed white blood cell count: 15,403 +/- 6,282/mm3, hemoglobin: 10.1 +/- 1.0 gm/dl, neutrophil: 59.2 +/- 13.7%, lymphocytes: 30.6 +/- 13.1%, platelet count: 456,3000 +/- 216,4000/mm3, and C-reactive protein 8.2 +/- 5.6 mg/dl. Patients with coronary artery dilation had a longer duration of diagnosis, higher incidence of atypical presentation, lower incidence of conjunctivitis, lower incidence of skin rash, lower incidence of extremity change, and lower C-reactive protein. The predictive value of coronary artery dilation based on the combination of atypical presentation, duration of diagnosis, and C-reactive protein was 81.2%. CONCLUSIONS: Kawasaki disease in infants is associated with a high incidence of atypical presentation and increased risk of coronary artery dilation. We suggest that in an infant with insufficient diagnostic criteria for Kawasaki disease, care should be taken to avoid missing atypical Kawasaki disease. Echocardiography is an important tool for diagnosis of atypical Kawasaki disease.

C-Reactive Protein↗

PubMed2001

Hyperbaric oxygen for carbon monoxide poisoning-induced delayed neuropsychiatric sequelae.

The clinical manifestations of delayed neuropsychiatric sequelae after carbon monoxide (CO) intoxication are variable. In addition, there is no specific therapy for these complications. Fortunately, these complications have occurred less frequently in recent years, probably due to the usage of hyperbaric oxygen (HBO) therapy. We report an 8-year-old boy who developed late psychiatric disturbances 2 days after full recovery of consciousness from initial CO intoxication. His neuropsychiatric symptoms included consciousness disturbance, motor dysfunction, chorea, aphasia and agnosias. He received HBO therapy at 2.0 barr for 60 minutes once a day for 7 consecutive days. Three weeks later, he was functioning normally with no neuropsychiatric symptoms. A literature review concluded that HBO may be effective in treating neuropsychiatric sequelae. Moreover, immediate administration of HBO during acute CO intoxication may prevent these complications.

Brain Diseases↗

PubMed2000

Seizure outcome after corpus callosotomy: the Taiwan experience.

From September 1989 to August 1996, we performed anterior corpus callosotomy in 83 patients. Unfortunately, 9 patients were lost to follow-up. Among the remaining 74 patients, 59 had Lennox-Gastaut syndrome (evolved from infantile spasms in 22), 9 had complex partial seizures with or without secondary generalized seizures, 1 had multifocal independent epileptogenic foci (MISF) syndrome, 3 had hemiconvulsion-hemiplegia-epilepsy (HHE), and 2 had infantile spasms. All cases were followed up for at least 2 years after surgery. The highest rate of significant improvement (more than 50% reduction in seizure frequency) was noted in the patients with generalized tonic-clonic seizures, 82.1% of whom experienced significant improvement, followed by those with generalized tonic seizures (76. 7%), atonic seizures (72.7%), myoclonic seizures (64.9%), atypical absences (58.6%), and complex partial seizure with or without secondary generalization (61.5%). Complete freedom from seizures was noted in 14 cases (18.9%). One patient had the anterior half of his right palm amputated following radial artery thrombosis complicated by insertion of an arterial line during anesthesia. Otherwise, there were no major postoperative complications except for brief mutism and multifocal jerks in some patients during the 1st postoperative week. Thus, we conclude that corpus callosotomy is a safe alternative treatment for all kinds of medically intractable seizures, especially generalized epilepsy.

Adolescent↗

PubMed2000

New mutations of the HPRT gene in Lesch-Nyhan syndrome.

Lesch-Nyhan syndrome is an X-linked recessive disorder involving the purine metabolism, with resultant hyperuricemia, choreoathetosis, self-mutilation, and profound neurologic dysfunction. A deficiency of the enzyme hypoxanthine guanine phosphoribosyl-transferase is responsible for the disease. The human HPRT gene is located at Xq26-27 and consists of 57 base pairs. At least 2,000 mutations throughout the HPRT gene coding region from exon 1-9 have been reported. Four patients from three Chinese families were diagnosed with Lesch-Nyhan syndrome according to the clinical and laboratory findings. DNA studies revealed the first family (Patients 1 and 2) had a missense mutation in exon 3 of the HPRT encoding region. This novel mutation occurs in the hot spot of the HPRT gene. The second family (Patient 3) was found to have a missense mutation in exon 8 of the HPRT gene. The third family (Patient 4) carried a mutation in the splicing region of intron 4 of the HPRT gene. All three mutations were de novo.

Base Sequence↗

PubMed2000

Comparison of 201Tl SPET and treadmill exercise testing in patients with Kawasaki disease.

About 4% of children with Kawasaki disease ultimately develop ischaemic heart disease. Therefore, the early detection, non-invasive monitoring and long-term follow-up of myocardial ischaemia are essential. We compared the sensitivity and specificity of 201Tl single photon emission tomography (SPET) and treadmill exercise in the detection of myocardial ischaemia in 23 patients (19 boys, 4 girls) with Kawasaki disease. They were divided into two groups according to the results of coronary angiography. Group I consisted of 11 patients with coronary abnormalities; Group II consisted of 12 patients with no coronary abnormalities. The sensitivity, specificity, false-positive and false-negative rates for detecting coronary arterial lesions were 72.7% (8/11), 58.3% (7/12), 38.5% (5/13) and 30% (3/10) for 201Tl SPET, and 45.5% (5/11), 100% (12/12), 0% (0/5) and 33.3% (6/18) for treadmill exercise, respectively. We conclude that 201Tl SPET is more sensitive than treadmill exercise for the detection of coronary arterial abnormalities, but that the specificity of treadmill exercise is better than that of 201Tl scintigraphy. Coronary artery lesions detected by coronary angiography have good concordance of ischaemic areas with perfusion defects detected by 201Tl SPET. When ischaemic findings on 201Tl SPET and/or positive treadmill exercise testing are noted, coronary angiography is strongly indicated to detect possible stenotic lesions in the coronary arteries.

Adolescent↗

PubMed2000

Pseudoaneurysm formation after infected modified Blalock-Taussig shunt: echocardiographic findings.

The modified Blalock-Taussig (B-T) shunt is well recognized as a palliative procedure for cyanotic congenital heart disease in infants. Pseudoaneurysm formation after a modified B-T shunt is a rare complication. We present the case of a 9-month-old girl with tetralogy of Fallot who had undergone a modified left B-T shunt at 5 months of age and developed a pseudoaneurysm after an episode of infective endocarditis as detected by echocardiography. Compression of the left bronchus, displacement of the mediastinum to right chest, and paralysis of the left hemidiaphragm were found. Magnetic resonance images, computed tomography scans, aortograms, and selective angiograms demonstrated the presence of a large pseudoaneurysm. The compression syndrome gradually disappeared after aneurysmectomy.

Anastomosis, Surgical↗

PubMed2000

The effect of breast- and bottle-feeding on oxygen saturation and body temperature in preterm infants.

From July 1997 to June 1998, 25 preterm infants (birth weight < 1800 g) were included in a prospective study to compare the clinical effects of breast- and bottle-feeding. Oxygen saturation, heart rate, respiratory rate, and body temperature were recorded every minute for 20 minutes during feeding periods. Eighty pairs of breast- and bottle-feeding sessions were observed at the chronological age of 9.3 +/- 4.3 (range = 2.1-25.3) weeks. Oxygen saturation and body temperature of the preterm infants were significantly higher when they were directly breastfed. There were 2 episodes of apnea (breath pause more than 20 seconds) and 20 episodes of oxygen desaturation (PaO2 < 90%) during bottle-feeding and none during breastfeeding. We conclude that breastfeeding is a more physiological feeding method for the preterm infant and bottle-feeding may be more stressful.

Apnea↗

PubMed2000

Congenital muscular dystrophy.

Congenital muscular dystrophy (CMD) is a rare heterogeneous disease found in the oriental population, especially the occidental type of CMD. We report a case of a one-year-old infant who presented with early onset hypotonia, muscular weakness, delayed motor development and normal intelligence. A muscle biopsy revealed dystrophic muscle fibers. A high creatine kinase (CK) level, mostly of the MM type, was also noted. Further study of brain images showed hyperintense lesions in the white matter area. The patient showed the clinical and laboratory findings characteristic of CMD, more likely to be of the occidental type. Further genetic or histopathologic studies, especially merosin investigation, are suggested for improved classification and prognosis prediction.

Humans↗

PubMed2000

Cytomegalovirus pericarditis with cardiac tamponade in a young infant.

The principal viruses implicated in pericarditis are enteroviruses. Cytomegalovirus pericarditis is quite rare and has been reported in immunocompromised patients with acquired immunodeficiency syndrome, malignant neoplasm or organ transplantation. We report a three-month-old male infant who suffered from cough and rhinorrhea for two weeks. He developed shortness of breath for three days, and fever for one day, prior to admission. Physical examination revealed tachycardia, tachypnea, pale conjunctiva, hepatomegaly, and a muffled heart sound without significant murmur. Chest radiography showed marked enlargement of the cardiac silhouette. Echocardiography demonstrated a large amount of pericardial effusion with impaired diastolic ventricular function. After pericardial drainage and supportive treatment, the fluid gradually disappeared. Viral culture of the pericardial fluid and serologic data confirmed a cytomegalovirus infection. Cytomegalovirus pericarditis should be included in the differential diagnosis of pericardial effusion in a young infant.

Cardiac Tamponade↗

PubMed2000

Chronic granulomatous disease: a case report.

Chronic granulomatous disease (CGD) is a rare inherited disorder caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex of phagocytic leukocytes. The leukocytes of the CGD patients cannot produce adequate amount of superoxide and other oxygen metabolites which are toxic to microorganisms. As a result, the phagocytes fail to kill the ingested microorganisms, especially those with catalase activity. Typically, CGD patients suffer from recurrent pyogenic infections starting from the first year of life. We report a young boy who had experienced recurrent perianal abscess, osteomyelitis and bacterial enterocolitis. Flow cytometric analysis revealed defects in the neutrophil respiratory burst pathway and defined the carrier state of his mother and younger sister. He received antimicrobial prophylaxis at our out-patient clinics and remained well at present. We try to make clinical physician keep in mind the diagnosis of CGD by presenting this typical case. In the meantime, we review the recent literature regarding the advances in diagnosis and management of CGD.

Animals↗

PubMed2000

Cardiac manifestations of fatal enterovirus infection during the 1998 outbreak in Taiwan.

BACKGROUND: An outbreak of hand-foot-and-mouth disease caused by enterovirus infection occurred in Taiwan in 1998 and more than 70 infants and children with fulminant courses died. We compared the cardiac manifestations of fatal cases with patients who survived the enterovirus infection. METHODS: A total 187 patients with enterovirus infection were treated at Taichung Veterans General Hospital between April and June 1998. Enterovirus infection was diagnosed by history, clinical features, polymerase chain reaction study and/or viral culture. Cardiac examinations including complete physical examinations, electrocardiography and echocardiography were performed on seven cases (group I) with or without central nervous system (CNS) involvement, 30 patients with CNS involvement (group II), and 150 patients without CNS involvement (group III). RESULTS: There were no significant differences in sex distribution, days of fever, heart rate, systemic blood pressure or time from the onset of symptoms to hospital admission among the three groups. All group I patients had features of acute congestive heart failure, pulmonary edema and neurologic signs except one who presented with right-sided heart failure and neurologic signs. The echocardiographic findings of group I were a lower fractional shortening, lower ejection fraction, and more severe and higher incidence of mitral regurgitation (p < 0.01) than in groups II and III, but there were no significant differences in end-systolic wall stress, left ventricular end-diastolic internal dimension and incidence of pericardial effusion among the three groups. CONCLUSIONS: We conclude that seven infants and children (group I) died due to either severe cardiomyopathy or encephalopathy. The possible pathogenesis of enterovirus infection leading to death is reviewed and analyzed.

Brain Diseases↗

PubMed2000

Invasive Streptococcus pneumoniae infections of children in central Taiwan.

We carried out a retrospective study on childhood invasive pneumococcal infections (IPI) diagnosed from the January 1990 through the April 2000 at a medical center in central Taiwan. Their clinical features, outcome of the patients and the resistance patterns of the isolates were analyzed. A total of 95 clinical isolates from 72 patients younger than 14 years of age were included in this study. Of these 72 patients, 51 had bacteremia, 28 meningitis, 14 bacteremic pneumonia, 12 pleural empyema, eight otitis media, four arthritis, three sinusitis, two periorbital abscesses, one deep neck infection, one psoas muscle abscess, one peritonitis, one urinary tract infection, and one cutaneous infection. Ancillary diagnostic tests, including Gram stain smears and latex agglutination tests, were applied and the sensitivities were 86.2% and 54.3%, respectively. The prevalence rate of penicillin nonsusceptible Streptococcus pneumoniae has increased dramatically since 1995 in central Taiwan, with rates of 5.6% and 74.1% before and after 1995, and the overall mortality rate was 20.8% and 53.3% respectively. Ten of 19 children (52.6%) with pneumococcal meningitis who survived had long-term sequelae.

Adolescent↗

PubMed2000

Influence of ventricular septal defect type on surgical results in children with tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) with subpulmonary ventricular septal defects (VSD) is relatively more common among Orientals than among Occidentals. This study assessed the influence of type of VSD on surgical results in children with TOF. METHODS: Fifty-one patients with TOF (age range, 14 months to 11 years, mean 40.7 months, median 30 months, 29 boys, 22 girls) who underwent total correction by a single surgeon between January 1992 and December 1995 were retrospectively studied. Of the 51 patients, 17 patients with subpulmonary VSD were classified as group I. The remaining 34 patients with perimembranous VSD were classified as group II. All patients underwent a combined transatrial and transpulmonary surgical approach. Early postoperative results were evaluated 7 to 10 days after surgery and late postoperative results were evaluated 1 year after surgery. RESULTS: There were no statistically significant differences between the two groups in age, sex, body weight, pulmonary to systemic flow ratio, pulmonary to aortic annulus diameter ratio or preceding palliative shunt or balloon pulmonary valvuloplasty. Nevertheless, group I patients had better oxygen saturation preoperatively (84.2 +/- 4.4% vs 80.3 +/- 7.6%, p = 0.037). During surgery, the transannular patch rate was higher in group I (100% vs 73.5%, p = 0.003). The cardiopulmonary bypass time, postoperative stay in the intensive care unit, duration of chest tube placement, early postoperative residual VSD rate, and early and late postoperative residual pulmonary stenosis rate were not significantly different between the groups. However, late postoperative residual VSD rate was 33.3% in group I and 6.45% in group II, which was statistically significant (p = 0.029). There were no significant differences in arrhythmia between the two groups. CONCLUSIONS: Patients with TOF with subpulmonary VSD had a better preoperative oxygenation, higher transannular patch rate during surgery and a higher incidence of late residual VSD than those with TOF with perimembranous VSD.

Cardiac Surgical Procedures↗