Biomedical subjects

W J Shian

Publications and source records attributed to W J Shian.

At least 37 records · Page 2Linked to original sources

PubMed1994

Congenital diaphragmatic hernia.

BACKGROUND: The management of congenital diaphragmatic hernia (CDH) remains a subject of controversy. With the objective to achieve a better future survival rate, clinical analysis of 14 cases during a 10-year period was done. METHODS: From 1983 to 1992, 14 cases with CDH were treated at Taichung Veterans General Hospital. The medical records of these patients were reviewed thoroughly. In the meantime, Mann-whitney U and Fisher's exact tests were used to analyze the factors predicting the outcomes. RESULTS: The overall mortality rate was 36%. Bochdalek hernia represented the most common defect in those patients who were operated upon (10/13, 77%). Ipsilateral pulmonary hypoplasia was present in five patients, four of whom died. Unilateral diaphragm agenesis was noted in two cases and both expired postoperatively. Initial arterial pH < 7.2, PaCO2 > 60 mmHg and early onset of symptoms may predict a poor outcome. There were no significant benefits from preoperative stabilization in those patients whose initial arterial pH values were lower than 7.2. CONCLUSIONS: CDH remains a major challenge for pediatricians and pediatric surgeons. Further studies are needed to deal with this frustrating disorder.

Female↗

PubMed1994

Epilepsy with grand mal on awakening.

BACKGROUND: Epilepsy with grand mal (GMA) on awakening is a benign generalized idiopathic epilepsy, occurring around the time of puberty. There have been few reports regarding Chinese children with GMA, thus this analysis of fourteen Chinese children with GMA who have been encountered in the past 10 years. METHODS: The medical and electroencephalographic (EEC) records of 14 Chinese children with GMA, 7 males and 7 females, were investigated retrospectively. RESULTS: The age of onset ranged from 9 to 15 years with a mean of 12 +/- 1.6 years. Six patients had previous history of febrile convulsion; four and three patients had positive family history for epilepsy and febrile convulsion, respectively. All patients had generalized tonic-clonic seizures on awakening and three of them had concomitant myoclonic jerks. The precipitating factors were sleep withdrawal (9), photostimulation (4), hyperventilation (3), fevers (2), and menstruation (2). The first EEG findings included 3-4 Hz generalized spike-wave complex (8/14), photostimulation activated (4/14), and hyperventilation activated (3/14). Valproate had been regularly administered orally for a duration of 1 to 8 years with a mean of 3.8 +/- 2.0 years up to time of study. Six (75%) out of eight patients relapsed about 6 months to 1 years after discontinuation of anticonvulsant. CONCLUSION: GMA may have some clinical relationship with other idiopathic generalized epilepsies, such as childhood absence epilepsy and juvenile myoclonic epilepsy. High-percentage relapse after drug discontinuation may be the rule for GMA patients, so long-term use of anticonvulsants may be needed.

Adolescent↗

PubMed1994

Brain electrical activity mapping in childhood absence epilepsy.

BACKGROUND: Brain electrical activity mapping (BEAM) studies related to children with epileptic seizures have been rarely reported. The authors wished to search for BEAM values in children with epilepsy, to better understand the changes in the cerebral field distribution of epileptiform events. METHODS: Electroencephalographies (EEGs) of 19 Chinese children with childhood absence epilepsy (CAE), but with normal intelligence, were studied prospectively and consecutively. Among them, 11 were boys aged from 6-12 years; 8 girls aged from 6-13 years. EEGs were simultaneously recorded on the Concerto (32 channels), and on the EEG machine, according to standardized recommendations. Frequency or spectral analysis was measured in terms of Band power (uv2), RMS (uv) and InstVolt (uv). Maps were also displayed simultaneously as color in their topographic distriution, with four-picture maximum. RESULTS: In comparing the four bands of background activities in subjects with eyes closed and open, in the different subgroups, there were no significant differences between boys and girls, nor between the group with epileptiform discharge events (EDE) and the group without EDE, except for the beta band in subjects with eyes closed of the EDE group. There was a significant increase of band power in the group treated with valproic acid, and an increase over the F3 in beta band, Fp1, F4 in theta band, F3, F4, P4, 02 in delta band, but no significant increase was found in alpha band. EDE was also measured and mapped and showed bilateral symmetry with a mid-frontal maximum. Root mean square (RMS) measurements of epochs, from the beginning of EDE to the end of EDE, showed significant increases in voltage over the F8 area. CONCLUSIONS: BEAM can clearly show cerebral field distribution, but also can employ statistical tests to give significance to the analyzed data. Furthermore, BEAM may be helpful for localization of the epileptiform discharges in EEG.

Adolescent↗

PubMed1994

Herpes simplex encephalitis in infants and children.

BACKGROUND: The estimated incidence of herpes simplex encephalitis (HSE) is 2 to 4 per million of the population per year, of whom one-third are neonates or older children and two-thirds, adults. As effective antiviral therapy improves the high mortality and morbidity for younger patients with HSE, early diagnosis has become important, especially in children. METHODS: Hospital records, neuroimages and electroencephalographic (EEG) findings of 10 Chinese infants and children with HSE were reviewed thoroughly over a 7-year period. Diagnosis of HSE was made via clinical setting of acute encephalitis with serological examinations of serum and cerebrospinal fluid (CSF). RESULTS: Fever, consciousness disturbance and seizures were the most common clinical presentations on admission. Most of the CSF findings were mild pleocytosis (< 200/cumm), red cells, slightly elevated protein level and normal ratio of CSF/serum glucose concentration. All patients had abnormal features, by brain computerized tomography, and most had low densities over the temporal and frontal regions. Five patients underwent follow-up brain magnetic resonance imaging and most showed features of brain atrophy and demyelinating changes over the temporal and frontal regions. Generalized slow background activity was the most common EEG finding. Four patients' EEGs showed periodic laterizing epileptiform discharge. Intravenous administration of acyclovir (30 mg/kg/day) for 10 days was the drug-of-choice in these patients. All patients but the three deaths had psychomotor or mental retardation and spasticity. Six patients had epileptic sequelae, and one patient had the sequela of amnesia. CONCLUSIONS: HSE remains a serious illness with high mortality and morbidity despite appropriate antiviral therapy. Adequate intravenous acyclovir therapy should be started as soon as possible. CSF examinations, EEG, serology, brain computerized tomography and magnetic resonance imaging may provide some clues of HSE, but negative findings should not deter or delay treatment in suspicious cases.

Adolescent↗

PubMed1994

Hypoxic-ischemic effect on infants and children with cyanotic congenital heart disease: clinical assessment of neurological examination and brain magnetic resonance images.

BACKGROUND: Progress in early surgery and intensive care have enhanced survival for more individuals with serious cyanotic congenital heart disease (CHD). Parents are understandably concerned about patients' brain function. Therefore, a study was undertaken of brain magnetic resonance images (MRI) in infants and children with cyanotic CHD, stressing the correlation between clinical neurological examination and brain MRI findings. METHODS: Sixteen infants and children with cyanotic CHD, 6 males and 10 females, were selected randomly from June 1991 to June 1993. Their ages ranged from 1 to 10 years, with a mean of 4.4 years. All patients underwent brain MRI and thorough clinical neurological examinations. RESULTS: Prominent and tortuous vessels over the Willis circle and basal ganglia were the most common brain MRI findings among patients with normal neurological examination (7/9), while ventriculomegaly (5/7) was the common findings for patients with abnormal neurological examination. Two patients who presented with low-grade fever and Babinski sign were found incidentally to have brain abscesses. CONCLUSIONS: It is not possible to make a conclusion from this preliminary report about the role which cyanotic CHD may play in the pathogenesis of brain insult, but the clinical neurological findings may provide guidance for the arrangement of brain MRI in infants and children with cyanotic CHD. Further larger scale, case-controlled study is needed to answer the controversial question whether cyanotic CHD per se causes brain insult.

Brain↗

PubMed1994

A long-term follow-up study of West syndrome.

BACKGROUND: Since the syndrome of infantile spasms, hypsarrhythmia and mental retardation was first delineated, there have been many publications about long-term prognosis of the West syndrome. This paper evaluates some of the early factors on which long-term prognosis may depend. METHODS: Patients with West syndrome were reviewed retrospectively according to the medical records as classification, response to initial medication regimen, prognosis and present neurological status. Seizure control, speech, psychomotor development, mentality, learning ability, behavior and motor handicap were followed over a mean period of 6.1 years. RESULTS: Thirty patients with complete hospital records were included in this study. Fourteen cases (46.7%) belonged to the symptomatic group, with a mean age of 3.4 months at onset. Four patients (13.3%) were cryptogenic type and a mean age of onset at 9.3 months. There were twelve cases (40%) in the doubtful group, whose mean onset age was 6.4 months. Of the symptomatic group, 92.8% patients had moderate to severe psychomotor retardation as compared to 25% in the cryptogenic group and 67% in the doubtful group; 35.7% of the symptomatic group patients suffered from motor handicap (spastic quadriplegia and hemiplegia as predominated), but only 8.3% of the doubtful group had motor handicap. CONCLUSIONS: The data collected showed that the following factors seemed to be related to better prognosis: later onset, normal to mild psychomotor delay upon diagnosis, and good seizure control.

Female↗

PubMed1993

Choledochal cysts: a nine-year review.

Hospital records of 24 Chinese infants and children (7 males and 17 females) with choledochal cysts were reviewed. The patients were aged 1 day to 17 years. Abdominal pain, palpable abdominal mass and jaundice, either alone or in combination, accounted for most of the initial presentations. Twenty-two patients were classified as type I with surgery, while the other two patients were type V without surgery. Ultrasonography is a rapid and accurate diagnostic method in the initial evaluation. Cyst excision with Roux-en-Y hepaticojejunostomy was the treatment of choice in this series. Chronic cholecystitis with or without inflammation of choledochal cyst was the most common pathological finding. Five patients underwent liver biopsies, showing four biliary cirrhosis and one portal fibrosis. So far, there is no evidence of malignancy in any patient.

Adolescent↗

PubMed1993

Juvenile and adult dermatomyositis among the Chinese: a comparative study.

Between 1983 and 1992, 11 children and 38 adults were seen at Taichung Veterans General Hospital with a definite diagnosis of dermatomyositis. Their clinical pictures, laboratory findings, courses and outcomes were compared. The mean age at diagnosis was 12 years and 50 years, respectively. Children had a higher female-to-male ratio (2.7:1 vs. 1.2:1) and a more acute onset, while adults had a higher incidence of malignancy and other connective tissue disease associations. Clinically, shawl sign was more common in the adults, whereas myalgia was more frequently seen in the children (0.05 < p < 0.1). Hemograms, serological parameters and immunological investigations showed no significant differences between the two groups. Elevated erythrocyte sedimentation rate and circulating immune complexes were found in over half of both groups of patients; however, their values bore no relationship to either disease activity or future outcome. Although no significant differences were demonstrated between the two groups of patients with regard to either clinical manifestations or laboratory findings, the disease entity seemed not to be the same because evidence of vasculopathy in muscle pathology was more prominent in the children, with endothelial swelling and necrosis the most common findings. Steroids had been used as the first choice of therapy in both groups of patients, followed by cytotoxic drugs and immunosuppressants. Children had a more favorable outcome compared to a 24% mortality rate in the adult group. Experience here recommends a complete cancer work-up in adults, especially for nasopharyngeal carcinoma among the Chinese. Various autoantibodies screenings may also be helpful for early detection of other associated connective tissue diseases.

Adolescent↗

PubMed1993

Joubert syndrome in Chinese infants and children: a report of four cases.

Four Chinese infants and children (3 females & 1 male), aged from five months to three years, are diagnosed with Joubert syndrome by clinical and radiological findings. The clinical presentations included panting respiration with apnea in the newborn period (4/4), psychomotor retardation (4/4) and ataxia (2/4). The ocular findings were strabismus (3/4), unilateral ptosis (2/4), jerky eye movement (1/4) and retinal atrophy (1/4). Associated cerebral anomalies were occipital encephalocele (1/4) and hypoplasia of corpus callosum (1/4). All four underwent electroencephalography, abdominal ultrasonography, auditory and visual evoked potential tests; results were all normal. Two patients underwent electroretinogram with normal findings. The brain magnetic resonance imagings of all four patients showed dysgenesis of cerebellar vermis. For children presenting with ataxia and psychomotor retardation, Joubert syndrome is a more obvious diagnostic choice, but it is also important to keep this unusual disorder in mind as a differential diagnosis of neonatal tachypnea with apnea.

Apnea↗

PubMed1993

Pseudomonas septicemia in infants and children: a retrospective analysis of 49 cases.

Reviewed 49 cases of Pseudomonas bacteremia which occurred in pediatric patients during an 8-year period showed the annual rate, per 1000 discharges, was 1.7. In most of the patients (73.5%), the disease was hospital-acquired. Male to female distribution was about 3 to 2. The average age of the 49 patients was 1.29 years, and 55.1% were infected at less than 2 months old. Overall mortality was 44.9%; among the mortalities, 63.6% were under two months of age. Clinical features were not characteristic, but the most common sign associated with this infection was fever (42.9%). Ecthyma gangrenosum occurred in only one patient. Respiratory tract and skin were the most frequent sources of the bacteremia. Polymicrobial bacteremia occurred in 18.4%. Patients with shock, pneumonia, inadequate antibiotic therapy or persistent neutropenia had a substantially poorer prognosis. Administration of combination therapy to patients with Pseudomonas bacteremia seemed to be superior to monotherapy for positive outcome.

Adolescent↗

PubMed1993

Central nervous system tuberculosis in infants and children.

Hospital records of 16 infants and children (9 males & 7 females) with tuberculous meningitis or tuberculoma were reviewed retrospectively over a 10-year period. Patients were aged from 5 months to 16 years, with a mean age of 7.2 years. The diagnoses were confirmed via a positive culture for Mycobacterium tuberculosis from cerebrospinal fluid (CSF) in 14 patients, and from brain tissue in 2. Most patients presented with fever (100%), conscious change (80%), vomiting (80%) and headaches (75%). Neurologically, meningeal and pyramidal signs were the most common findings. Lymphocytic CSF pleocytosis with hypoglycorrhachia, increased protein, and decreased CSF/serum glucose ratio were the major CSF findings in our patients. Hyponatremia was present in 70%. Brain computerized tomography showed hydrocephalus (87%), basal exudate (50%), ischemic infarction (37%) and tuberculoma (12%). Two patients (12%) expired and 10 (71%) of the surviving patients had neurological sequelae. In summary, the characteristic CSF findings and hydrocephalus (87%) seemed to be sensitive clues supporting early initiation of antituberculous drug therapy and thorough investigation as reflected in this series.

Adolescent↗

PubMed1993

Clinical studies of Henoch-Schönlein purpura in Chinese children.

Over a 9-year period from 1983 to 1992, 50 patients with Henoch-Schönlein purpura were admitted to the Department of Pediatrics, Taichung Veterans General Hospital. There were 33 boys and 17 girls, with a male to female ratio of 2:1. Their ages ranged from 2 to 14 years with a mean of 6.3 years. Seventy percent of the patients were over the age of five. The months of peak incidence were October to December (48%). Fifty-four percent of the patients had evidence of preceding infections. The main clinical features were skin rashes (100%) followed by gastrointestinal symptoms (66%), joint symptoms (58%), soft tissue edema (30%) and renal involvement (30%). Hemograms showed leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate in about half of these patients. Serological examinations were nonspecific; anti-streptolysin O titers were elevated in 40% of the patients. Forty-two percent of our patients had elevated serum IgA levels which bore no relationship to the symptomatology and duration of the illness. One patient had a complication of intussusception requiring surgical reduction. Another patient who had renal involvement progressed to end-stage renal disease. Eighty-two percent of our patients received prednisolone therapy at a dose of 1-2 mg/kg/day, with good response. Seventy-two percent of the patients recovered completely within one month after the onset of the malady. The overall prognosis remains good.

Adolescent↗

PubMed1993

Congenital tracheal stenosis: a report of two cases.

Congenital tracheal stenosis (CTS) is a rare, potentially lethal condition. Two cases were diagnosed in this hospital between 1986 and 1992. Diagnosis was confirmed by endoscopy and radiography. These two cases were noted to have symptoms of airway obstruction after an infectious process. Their outcomes were quite different. The first patient, a victim of CTS diagnosed at two years old, still lives well and has been asymptomatic for subsequent six years, though he has received no surgical intervention. The second patient, a three-month-old female, exhibited an exacerbation after bronchoscopy and died in spite of intensive care. Prompt recognition of symptoms and a thorough evaluation of airway, as well as other anomalies, is essential for proper management of children with CTS. The diagnostic modalities, management and risk factors are discussed.

Child, Preschool↗

PubMed1993

Epstein-Barr virus encephalitis: clinical observations in nine Chinese children.

Nine Chinese children (four males, five females) in whom serology showed Epstein-Barr virus infection were studied retrospectively. They were from 1.5 to 14 years of age. Various symptoms and signs, including disturbance of consciousness, visual hallucination, cranial neuropathies and sphincter dysfunction, led to initial clinical impressions of encephalitis, encephalomyelitis and neuro-degenerative disease. Electroencephalography showed focal spikes over the frontal, occipital, temporal and parietal areas. The patients underwent brain computed tomography and/or magnetic resonance imaging, and three of them underwent HMPAO SPECT. Eight patients appeared normal neurologically during follow-up, and the single patient who had spastic quadriparesis improved gradually. Though visual hallucination is not a specific presentation, it is a peculiar picture of Epstein-Barr virus encephalitis. Epstein-Barr virus encephalitis seemed to be self-limiting without specific treatment in this group.

Adolescent↗

PubMed1993

Abnormal mitochondria in Rett syndrome: one case report.

A 6-year-9-month-old girl with the characteristic features of Rett syndrome is reported. Clinically, she had microcephaly, psychomotor arrest, deterioration of communication, autistic behaviour, loss of language development, gait apraxia and stereotyped hand washing movement. Amino acid and organic acid analysis were normal. An abnormal rise in serum lactate was noted 120 minutes after oral glucose loading. Muscle biopsy was performed and there was no specific finding noted under light microscope. Electron microscopic evaluation revealed mild accumulation of mitochondria at subsarcolemmal area with abnormal tubular cristae. The cause of Rett syndrome remains obscure. Several articles concerning abnormal mitochondrial morphology or respiratory enzymes have been reported. The exact pathogenesis requires further investigation.

Child↗

PubMedDate not supplied

Oral glucose lactate stimulation test in mitochondrial disease.

We selected 23 patients with neurologic diseases, including 15 boys and 8 girls from 1 month to 10 years of age, who were divided into mitochondrial and nonmitochondrial disease groups. All patients were required to fast and rest for at least 4-10 hours before the test. Glucose was administered orally using a 50% glucose:water solution at a dose of 1.75 mg/kg. Blood samples then were drawn from a retained heparinized venous line at 0, 30, 60, 120, and 180 min and tested for lactate and glucose levels. Of the mitochondrial disease group, 10 of 11 patients had an upward sloping curve of lactate metabolism; the one who had a flat line response was a patient who suffered from a free-fatty-acid metabolic defect presenting with a recurrent Reye-like syndrome. There was a relatively flat line response in the nonmitochondrial disease group except in a patient with alternating hemiplegia whose symptoms responded well to flunarizine therapy. A significant increase in blood lactate content at 60 min after glucose loading occurred in the mitochondrial disease group, in contrast to that of the nonmitochondrial group. Sensitivity and specificity of a 5 mg/dl increase in blood lactate concentration at 60 min was 72.7% vs. 91.7% Moreover, all 4 patients whose blood lactate increased by 13 mg/dl at 60 min were in the mitochondrial disease group.

Biopsy↗

PubMedDate not supplied

Late infantile form metachromatic leukodystrophy: report of one case.

Metachromatic leukodystrophy (MLD) is a neurodegenerative disorder with autosomal recessive inheritance, in which cerebroside sulphate (sulphatide) accumulates in the central and peripheral nervous systems due to a deficiency of arylsulphatase A. This article presents a 2-year-old boy who had occasional shortness of breath, horizontal nystagmus and unstable gait for 3 months prior to the entry. He was admitted to our hospital due to shortness of breath, frequent apnea, generalized hypotonia and conscious disturbance. The lumbar puncture, brain CT scan, serum amino acid analysis, urine organic acid assay and nerve conduction velocity of lower extremities all showed negative findings. The electron microscopic finding of muscle elicited lipid deposition. The auditory brainstem response showed bilateral impairment. The routine EEG revealed diffuse slow waves. The brain MRI showed widespread low signals over the white matter of bilateral frontal and parietotemporal areas of the cerebral hemispheres, as well as the white matter of the bilateral cerebellar hemispheres, and the brain stem in the T1-weighted image corresponding the high signals in T2-weighted image. The blood leukocyte lysosomal enzyme activity test revealed arylsulphatase A deficiency. Rapid progressive neurological deterioration was noted since admission. Unfortunately, the patient expired due to respiratory failure in the final.

Bone Marrow Transplantation↗

PubMedDate not supplied

[Infantile bilateral necrosis of the striatum of corpus: report of four cases].

The designation of "Infantile Bilateral Striatal Necrosis" (IBSN) was first given by Friede in 1975. However, this unusual condition was first described by Paterson and Carmichael in 1924. The disease is rare with uncertain etiology. The clinical picture includes choreoathetosis, abnormal eye movements, seizures and mental dullness. These circumstances often follow symptoms such as fever, vomiting and impaired consciousness. The final diagnosis is confirmed by pathological examination, which reveals symmetrical degeneration of bilateral basal ganglia. With present technology IBSN can be well demonstrated in the brain Ct scans or MRI scans nowadays. This article reports four cases with clinical manifestations which had appeared before the age of one year. Three cases had prodromal upper respiratory tract infection symptoms with vomiting, while seizure and impaired consciousness ensued. One case had several bouts of pneumonitis followed by seizures, impaired consciousness and abnormal eye movement. Brain sonogram of one of these cases showed hyper-echoic basal ganglia, while CT scans or MRI scans revealed symmetrical hypodensity or signal change over bilateral basal ganglia, respectively. All of these led to a bedridden life. These four cases are reported based on their clinical presentations and brain imaging findings, in spite of the absence of pathological confirmation. Some of the literature are also reviewed. To sum up, IBSN should be kept in mind in the differential diagnosis of symmetrical bilateral basal ganglia lesion after the exclusion of other disorders such as neurometabolic disorders, central nervous system infection, carbon monoxide intoxication, hypoxic-ischemic encephalopathy, tumors and cerebrovascular disorders etc.

Corpus Striatum↗