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Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 55 records · Page 3Linked to original sources

MRI of Joubert's syndrome.

OBJECTIVE: To report five cases of the rare Joubert's syndrome. SUBJECTS: All five cases were studied by 1.0-Tesla MRI. All the patients showed typical clinical manifestations of Joubert's syndrome including neonatal respiratory abnormalities, developmental delay, ataxia, retinal atrophy and nystagmus. RESULTS: The T1WI of MRI showed characteristic MRI features of Joubert's syndrome including dilatation of the fourth ventricle with some appearing bat-wing shaped, elongation and stretching of the superior cerebellar peduncles, dysphasia of the vermis, widening of the foramen of Magendie and the posterior cistern. One case was associated with encephalomeningocele. CONCLUSIONS: MRI can provide characteristic findings of Joubert's syndrome and confirm the clinical diagnosis.

Brain↗

Leigh syndrome with progressive ventriculomegaly.

A 6-month-old female infant was diagnosed with Leigh syndrome after an abnormal muscle specimen was obtained and after magnetic resonance imaging demonstrated characteristic cranial lesions. She presented with episodic hyperventilation, myoclonus, ophthalmoplegia, hypotonia, and elevation of lactate in the cerebrospinal fluid and blood. A series of cranial ultrasounds revealed progressive ventricular enlargement before the typical lesions were detected by magnetic resonance imaging. Myelin destruction is believed to play an important role in the early stage of Leigh syndrome. Ultrasonography may provide a convenient way to document changes in brain that provide early suspicion of Leigh syndrome.

Brain↗

Tc-99m HMPAO brain SPECT findings in pediatric viral encephalitis.

Two children were diagnosed with viral encephalitis, due to Epstein-Barr virus infection in one case and to herpes simplex virus infection in the other. Tc-99m HMPAO brain SPECT was arranged to detect changes in regional cerebral blood flow (rCBF) secondary to viral encephalitis. During the acute episode, Tc-99m HMPAO brain SPECT showed that the two cases had increased rCBF. After the acute episode, follow-up brain SPECT was arranged 6 months later. The rCBF in one case was restored to normal on the second brain SPECT, and that in the other case was decreased. The child with normal rCBF in the follow-up brain SPECT had better learning ability and intelligence than the other child with decreased rCBF.

Brain↗

Varicella-zoster virus infection in children with malignancy.

BACKGROUND: Immunocompromised children are potentially threatened by infections, among which, the highly contagious chickenpox infection is the most common. In the past six months, there has been a spate of five chickenpox infections in children with malignancy, all of whom were receiving chemotherapy at that time. METHODS: The cases of 17 children with malignancies, who suffered from varicella-zoster infection during a period of chemotherapy at Taichung Veterans General Hospital were reviewed. RESULTS: The diagnoses of their neoplasms were 12 acute lymphoblastic leukemia (ALL), 2 lymphoma, 3 solid tumors. The mean age was 6.8 +/- 4.0 year-old (range 3 to 15 year-old). The average duration from chickenpox skin eruption to admission was 3.3 +/- 1.8 days. Four patients suffered from abdominal pain and three of them died soon; three of them suffered from back pain and one died later. Seven of these 11 patients had impaired liver function (GOT > 45 U/L), of whom 4 died later. There were seven patients with pneumonitis, of whom five died later. Among 12 patients with ALL, 3 had absolute lymphocyte counts (ALC) < 500/mm3, but only 1 died later; 9 had ALC > 500/mm3, of whom 4 had pneumonitis, and all died later. Four patients developed disseminated intravascular coagulopathy, and three of them died later. Seven patients were prescribed acyclovir within three days after first skin eruption, none of these died. Ten patients were prescribed acyclovir three days or more after first skin eruption and five of them died later. Five patients were prescribed intravenous immunoglobulin (IVIG) within three days after first skin eruption, and none of them died; of the seven patients prescribed IVIG three days or more after first skin eruption, three died later. CONCLUSIONS: Abdominal pain and disseminated intravascular coagulopathy (DIC) were signs of visceral dissemination. Severe liver function impairment, pneumonitis and DIC were the principal causes of death. Early administration of acyclovir and intravenous immunoglobulin (IVIG) can probably effectively prevent the dissemination of varicella-zoster virus (VZV). While varicella-zoster immunoglobulin (VZIG) was unavailable, IVIG was still valuable in treating VZV infection.

Acyclovir↗

Right ventricular myxoma in a 12-year-old child: a case report.

Cardiac myxoma especially of the right ventricle, is rare in children. A 12-year-old boy with right ventricle myxoma is reported. His heart murmur was found incidentally when he visited the Outpatient Department (OPD) because of upper respiratory infection symptoms. There was no exertional dyspnea, no palpitation, no syncope and no skin lesion. The echocardiogram revealed a heart tumor of about 4 x 3 cm in the right ventricle (RV) extending to the right atrium (RA). The tumor grew from the interventricular septum, resulting in an RV outlet and inlet obstruction. Magnetic resonance image (MRI) revealed a well-defined mass in the RV ventricle, with good enhancement after injecting a contrast medium. The patient received right atriotomy and total excision of the tumor with repairing of the interventricular septum base. Pathology revealed myxoma with gelatinous whitish color and central hemorrhage. At post-operative follow-up, the patient's condition was good.

Child↗

Guillain-Barre syndrome in infants and children.

BACKGROUND: Guillain-Barre syndrome (GBS) occurs at all ages, and population-based studies give it a crude average annual incidence rate ranging from 0.4 to 1.7 per 100,000 population. This retrospective study is performed for fifteen Chinese infants and children in light of the fact that GBS infrequently occurs during childhood years. METHODS: Hospital records of fifteen Chinese infants and children, i.e. eleven males and four females, with GBS were included. Their ages ranged from one to sixteen years old, with a mean average of 8.5 years. Their performances were graded on a scale from 0 to 6 according to the GBS Study Group. Their performances were graded under categories of Grades 2 (2/15), 3 (2/15), 4 (8/15) and 5 (3/15). RESULTS: Seven patients (46.7%) had preceding viral illness, occurring from seven to fourteen days prior to admission, with a mean average of twelve days. In addition to weakness and areflexia, musculoskeletal pain and subjective paresthesia were the most common presentations upon admission. The later the cerebrospinal fluid (CSF) was examined, the more cases had CSF features of increased protein level with no or mild pleocytosis. Eleven patients underwent serological examinations, and seven of their tests yielded positive for Epstein-Barr virus in five and Mycoplasma pneumoniae in two. Seven patients underwent electromyography and nerve conduction velocity measurement, all exhibiting the characteristic features of demyelination. Twelve patients had supportive treatment, and three of them required mechanical ventilation. Intravenous gamma globulin was administered in two patients, and oral prednisolone was administered in one patient. It took one to six months with a mean average of 3.4 months for patients' performance returning to Grade 0. All of the patients survived without residual disability. CONCLUSIONS: Some infectious pathogens could play a role in the pathogenesis of GBS. Furthermore, complete recovery may be the rule for most infants and children with GBS.

Adolescent↗

Clinical study of epileptic children with history of febrile convulsion.

BACKGROUND: Clinically, some epileptic syndromes have been linked to febrile convulsions (FC), but the exact relationship between FC and later epilepsy remains a mystery. METHODS: Detailed histories of 81 Chinese children among 1950 pediatric epileptics with a history of FC were obtained retrospectively. The clinical courses and risk factors were analyzed. RESULTS: According to their epileptic patterns, the children were divided into a generalized group (Group G, 45/81), a partial group (Group P, 30/81) and those with severe myoclonic epilepsy in infancy (6/81). Patients of Group P had an earlier age of onset of FC with a larger number of risk factors than those of Group G (p < 0.01), and patients of Group G had a shorter interval from the last FC to later epilepsy than those of Group P (p < 0.01). More risk factors of FC were present in children of Group P; these included especially focal seizures, prolonged duration and retarded psychomotor development (p < 0.01). CONCLUSIONS: For group of epileptics in this study, genetic factors might predispose for the expression of FC and later epilepsy, and anticonvulsant prophylaxis for FC seemed to be unnecessary.

Child, Preschool↗

Dural arteriovenous malformation with symmetrical calcification of the basal ganglia: a case report.

Dural arteriovenous malformations associated with symmetrical calcification of the basal ganglia are rare in children. This report concerns a 22-month-old female infant who was admitted with the problem of acute onset of status epilepticus. Physical examination revealed a grade II/VI heart systolic murmur over the left sternal border, and engorged scalp veins. Neurologically, left side hemiparesis, brisk deep tendon reflexes, bilateral presence of Babinski sign and ankle clonus were present. Brain computed tomography without contrast medium showed cortical atrophy and symmetrical calcification of the basal ganglia. Brain magnetic resonance imaging showed a signal-voided tortuous structure over the right parietal region. Cerebral angiography disclosed a dural arteriovenous malformation, located over the right parietal region, which had two major feeding arteries from the branch of the anterior cerebral artery and the parietal branch of the middle cerebral artery, and had direct venous drainage into the superior sagittal sinus. The patient received craniotomy with ligation of feeding arteries. She became seizure-free, and her neurological deficits improved gradually.

Basal Ganglia↗

Viral encephalitis in children: detection with technetium-99m HMPAO brain single-photon emission CT and its value in prediction of outcome.

PURPOSE: To assess the role of regional cerebral blood flow measured by technetium-99m hexamethyl propyleneamine oxime (HMPAO) brain single-photon emission CT (SPECT) in viral encephalitis of children. METHODS: Eighteen children diagnosed as having viral encephalitis (12 Epstein-Barr virus, 4 herpes simplex virus, and 2 Japanese B virus) underwent Tc-99m HMPAO brain SPECT and brain MR and/or CT. RESULTS: During the acute episode, 4 (22%) of the 18 patients had localized abnormality on brain MR and/or CT. Tc-99m HMPAO brain SPECT in the acute phase showed that 17 (94%) of the 18 patients had increased regional cerebral blood flow and 1 (6%) of the 18 children had a normal brain SPECT. Follow-up brain SPECT was performed at least 15 days after the acute episode. In 17 patients with abnormal first brain SPECT, 12 (71%) had normal second brain SPECT and 5 (29%) had decreased regional cerebral blood flow. The group of patients with normal regional cerebral blood flow on the follow-up brain SPECT had a better outcome than the group of patients with decreased regional cerebral blood flow. CONCLUSIONS: (a) The Tc-99m HMPAO brain SPECT was abnormal more often than CT or MR in children with acute viral encephalitis and provided better location. (b) In acute episodes of encephalitis, most patients showed locally increased regional cerebral blood flow. (c) After acute episodes, the regional cerebral blood flow returned to normal in most cases. (d) A normal Tc-99m HMPAO brain SPECT in the subacute phase usually indicates a good clinical outcome (no neurologic defect) 1 year after the acute illness in children with viral encephalitis.

Adolescent↗

Clinical observation of neonatal gastrointestinal perforation.

BACKGROUND: Gastrointestinal perforation is an infrequent occurrence in neonates. Experience in its management is presented, while also attempting to analyze the factors affecting outcome. METHODS: From 1983 to 1992, 31 neonates with gastrointestinal perforation were treated at Taichung Veterans General Hospital. The medical records of these patients were reviewed thoroughly. In the meantime, Mann-Whitney U and Yates' correction chi square tests were used to analyze the factors predicting the outcomes. RESULTS: There were 21 males and 10 females, among whom 16 were premature births. The median age at diagnosis was 5 day; half (16/31) had occurred during the first 5 days of life. Abdominal distension was the most common manifestation (87%). Hemograms at admission showed leukopenia in 32% (10/31) and thrombocytopenia in 40% (8/20) of the patients. Metabolic acidosis was present in 46% (13/28) of the patients. Peritoneal fluid and preoperative blood cultures were positive in 71% (17/24) and 50% (13/26) of the patients, and both of them had Gram-negative enterobacteriacea as the most common pathogen. The overall mortality rate was 58%. The highest mortality rate was associated with gastric perforation (100%), followed by small bowel (50%) and colon (50%) perforations. The predominant cause of perforation was necrotizing enterocolitis (14/31), with the most common site the terminal ileum (16/29). Four patients with necrotizing enterocolitis had multiple perforations. Others who underwent surgery showed single perforation. The major cause of death in those patients who received operation was sepsis (8/13). An initial arterial pH value higher than 7.25, and surgical procedure performed within two days after disease onset may predict a favorable outcome (p < 0.01). CONCLUSIONS: Gastrointestinal perforation is a life-threatening complication in neonates. A better survival rate can be obtained by cooperation among neonatologists, pediatric surgeons and the nursing staff taking care of these high risk babies.

Chi-Square Distribution↗

Childhood absence epilepsy.

BACKGROUND: Childhood absence epilepsy (CAE) is a relatively rare form of idiopathic generalized epilepsy, occurring in previously normal children with a strong genetic predisposition. The annual incidence of CAE has been estimated at 6.3/100,000 to 8/100,000 children aged 0-15 years. METHODS: Medical and electroencephalographic records of 50 Chinese children with CAE were reviewed. RESULTS: There were 22 males and 28 females. The age of onset ranged from 3 to 12 years with a mean of 6.9 +/- 2.4 years. Six children (12%) had positive familial epileptic history and 11 patients (22%) had a past history of febrile convulsion. Electroencephalographically, there were posterior delta rhythm (6/50), frontocentral (1/50), and centrotemporal (1/50) spikes in addition to the characteristic 3 Hz spike and wave complex. All patients received regular or irregular treatment with valproate. Sixteen patients (32%) evolved to generalized tonic-clonic seizure (GTCS) with a mean of 2.1 +/- 1.6 years of lag from stopping valproate. The patients with GTCS had significant late onset of age, a longer active period, and longer treatment duration than those without GTCS (p < 0.01). There were significantly more patients with irregular treatment evolving to GTCS than those with regular treatment (p < 0.01). None of the patients with posterior delta rhythm evolved to GTCS. CONCLUSIONS: Regular treatment with valproate is the modality of choice in the management of CAE. Posterior delta rhythm seems to be a good prognostic factor electroencephalographically.

Child↗

Congenital duodenal web in late infancy and childhood: a report of two cases.

Congenital duodenal webs are rare lesions, usually detected during early infancy because of signs of high intestinal obstruction. The occasional patient escapes both symptoms and detection until adolescence or adulthood. This report concerns two cases of congenital duodenal web at different ages and with different clinical manifestations. Case 1, a six-month-old male, was admitted because of abdominal distention and vomiting. Case 2, a 13-year-old boy, was referred here for further evaluation of recurring seizure attacks, elevated blood urea nitrogen and creatinine and hyponatremia. Duodenotomy and excision of the web performed for both patients. Complete amelioration of all symptoms was then observed at Outpatient Clinic follow-up for one year.

Adolescent↗

Leukemoid reaction in a child with appendiceal abscess: a case report.

A case of appendiceal abscess presenting leukemoid reaction is reported. A seven-month-old male infant was admitted because of fever and diarrhea. Anemic conjunctiva and mild abdominal distension were the only physical findings on arrival. Progressive leukocytosis with left shift maturation was noted during hospitalization. The maximal white blood cell count was 178,000/cumm, with a high leukocyte alkaline phosphatase score. On the fourth day of admission, a palpable right lower quadrant abdominal mass with local tenderness was found. Appendiceal abscess was diagnosed by both ultrasonography and computed tomography. The infant received triple antibiotic therapy consisting of ampicillin, gentamicin, and clindamycin for 10 days; the mass disappeared two weeks later. As appendiceal abscess is a rare occurrence in infants and its production of a leukemoid reaction is even rarer, the case described here may cause a diagnostic challenge, and deserves attention from clinicians.

Abscess↗

Juvenile myoclonic epilepsy.

BACKGROUND: Recognition of the significance of juvenile myoclonic epilepsy (JME) in the English-language neurological literature is relatively new. There are many factors responsible for delay in diagnosis of JME, including lack of familiarity with the syndrome, failure to elicit a history of myoclonic jerking and absence or generalized tonic-clonic seizures predating myoclonic jerks in some patients. METHODS: The medical and electroencephalographic (EEG) records of seven Chinese children with JME, four males and three females, were reviewed. RESULTS: The age of onset ranged from 10 to 14 years with mean 11.8 +/- 1.6 years. The precipitating factors were sleep deprivation (5/7), photostimulation (3/7), hyperventilation (2/7) and menstruation (1/3). Childhood and juvenile absence epilepsies predated JME in three and one patients, respectively. All patients had concomitant grand mal on awakening. Generalized 3-4 Hz polyspikes-wave complexes occurred in all patients, and two patients had additional 3-4 Hz spike-wave complexes. These activities were provoked with photic stimulation (3/7) and hyperventilation (2/7). All patients were treated with valproate for more than four years, and relapse occurred six months to one year after discontinuation of valproate. CONCLUSIONS: JME is a benign generalized epilepsy with a strong genetic basis. Valproate is the drug of choice up to now, but long-term (life-long) medication may be needed.

Adolescent↗

Salmonella infections in infants and children.

BACKGROUND: Salmonella is an important pathogen for infants and children of which multiply resistant strains were recently reported. Our interest was the annual variation of clinical manifestations, serotype and the sensitivity rate for antibiotics during the past decade. Increased incidence of bacteremia in infants is reported. In order to find the predisposing factors of patients with acute gastroenteritis complicated with bacteremia, we conducted a retrospective analysis. METHODS: We reviewed the records of documented patients who had positive culture for Salmonella in our Pediatric Department from September 1982 to September 1992. Those patients with acute gastroenteritis were divided into two groups: I, those complicated with bacteremia and II, non-bacteremic cases. Then we compared the age, body temperature, severity of clinical symptoms, white blood cell count and Salmonella serotype of these two groups. RESULTS: There were 180 cases of Salmonella infection in our Pediatric Department during the past decade. The mean age was 14.80 months. Most (88.9%) patients were under two years old and 146 subjects were victims of acute gastroenteritis. Six cases had infection of the central nervous system (CNS). Five cases presented typhoid fever. Of three mortal cases, two died from meningitis with sepsis and one was a victim of malignant lymphoma and septic arthritis complicated with sepsis. Salmonella group B was the most common pathogen. There were no cases of typhoid fever after 1989. The sensitivity to ampicillin, chloramphenicol, and ceftriaxone for group B were 25.7%, 26.1% and 100% respectively. Multiply resistant Salmonella species were not present in our cases. The severity of clinical symptoms varied in significantly between those who had acute gastroenteritis with bacteremia (Group I) and those who had acute gastroenteritis without bacteremia (Group II). However, there was a significant distinction in the average age of the two groups (P = 0.005). CONCLUSIONS: Although the annual number of cases did not decrease in recent years, the number of cases of typhoid fever did decrease. Most patients were infants and acute gastroenteritis was most common. In our series, the relative sensitivity for ampicillin, chloramphenicol was small over the past decade. Multiply resistant Salmonella species were not found, which may be due to the small sample size.

Acute Disease↗

Total anomalous pulmonary venous return: a clinical observation of 36 cases.

BACKGROUND: More and more infants with total anomalous pulmonary venous return (TAPVR) has been detected in this hospital (Taichung Veterans General Hospital) in recent years as new diagnostic methods have developed. Several of these methods have been compared to assess their influence on accuracy of diagnosis and the perceived incidence of TAPVR: METHODS: A retrospective study was made of 36 patients, hospitalized with TAPVR between January, 1983 and June, 1993. All 36 had received 2D echo with color Doppler. Fifteen patients were diagnosed by cardiac catheterization. For the 21 patients who did not receive cardiac catheterization, diagnosis was proved in 16 by operation; in 3, by autopsy; another 2, by 2D echo with color Doppler and magnetic resonance imaging (MRI). RESULTS: Twenty-five patients were boys and 11, girls. Their ages ranged from 1 day to 6 years with a mean of 3.6 months. Twelve patients were supracardiac type (33%), 18 cardiac type (50%), 5 infracardiac type (14%); and only 1 was mixed (3%). Nine of the 11 girls (82%) were cardiac type, while only 9 (36%) of the 25 boys were cardiac type (P < 0.05). In 4 (80%) of 5 infracardiac-type patients the pulmonary venous returns were obstructed, while in only 8 (26%) of 31 non-infracardiac type patients was there evidence of obstruction (P = 0.06). The 11 patients who did not receive operations died. Operation mortality for isolated TAPVR was 0% (0/9) for the nonobstructed-nonrestrictive type patients, and was 50% (5/10) in the other two types (P < 0.05). Operation mortality in those younger than 30 days was 66.6% (2/3), and in those older than 30 days was 18.8% (3/16) (P = 0.15). Of 10 patients associated with complex heart diseases, 9 (90%) were common atrioventricular canal. Of the 18 patients diagnosed before July 1990, 15 patients received cardiac catheterization; of the 18 diagnosed after August 1990 (13 of whom were simple TAPVR), none had catheterizations. Eight patients received MRI which revealed good images of the pulmonary veins. CONCLUSIONS: Both echo with color Doppler and MRI are non-invasive tools which can make definite diagnoses of TAPVR: These new methods of diagnosis reveal that this disease is not so rare as was once thought. Cardiac catheterization adds little except risks and costs to diagnosis of simple TAPVR:

Child↗