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Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 73 records · Page 4Linked to original sources

Acute transverse myelitis in children: clinical analysis of seven cases.

BACKGROUND: The reported annual incidence of acute transverse myelitis (ATM) is 1 per 3.4 million. Because of its rarity, we performed this retrospective clinical investigation. METHODS: Hospital records of seven Chinese children with ATM were reviewed thoroughly. The diagnosis of ATM were made clinically with or without neuroimages. RESULTS: Five males and two females were included, of age from three to sixteen years with a mean of 8.6 years. The common presentations included weakness of lower extremities, acute flaccid paralysis, urinary distention, constipation, hyporeflexia, sensory impairment and paresthesia. Slightly elevated protein level in cerebrospinal fluid was noted. Mycoplasma pneumoniae, varicella/zoster virus and Epstein-Barr virus infections were confirmed serologically in one, one, and two patients, respectively. All patients received supportive treatment. The duration required to reach peak severity from onset of illness ranged from six to forty-eight hours. All but one patient recovered, and one to six months were required to achieve maximum improvement. CONCLUSIONS: Some infectious pathogens may play a role in the pathogenesis of ATM. In general, the prognosis of childhood ATM is good.

Acute Disease↗

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↗

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↗

Infective endocarditis in infants and children during the past ten years.

BACKGROUND: Infective endocarditis occurs infrequently in the general pediatric population, but is often associated in patients with underlying heart disease. Even today it remains a serious threat leading to relevant mortality in all pediatric groups, in spite of better antibiotic treatment and prophylactic measures for endangered persons. This retrospective analysis reports experience here with infective endocarditis in children. METHODS: The clinical and laboratory findings in 28 children with 30 episodes of infective endocarditis, from July 1984 to June 1993, were reviewed retrospectively. RESULTS: Twenty-one children (75 percent) had congenital heart disease, three (11 percent) had rheumatic heart disease and four (14 percent) had no underlying cardiac abnormality prior to presentation. Twenty-six (87 percent) of the 30 episodes had positive blood cultures. The most common organism isolated was streptococcus viridans (14 percent), followed by staphylococcus aureus (7 percent). Echocardiography identified vegetation in 21 of 28 (75 percent) patients. Twenty children were successfully treated medically. Four developed complications requiring surgery, of whom two died. A total of six children (21 percent) died of the disease or its complications. There were a high case fatality rate for children below the age of two years (75 percent), and children without an underlying cardiac abnormality (50 percent). CONCLUSIONS: It was concluded that patient survival is dependent on several factors which include early diagnosis, appropriate antimicrobial therapy and aggressive surgical intervention.

Adolescent↗

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↗

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↗

Meconium peritonitis in neonates.

BACKGROUND: Meconium peritonitis is a rare occurrence in neonates. Experience in its management is presented, while also attempting to compare present results with previous reports. METHODS: Between 1983 and 1993, 10 patients of meconium peritonitis were studied retrospectively. Clinical pictures, image studies, course and management were reviewed thoroughly. RESULTS: There were seven males and three females, with two premature births. None of these patients had associated cystic fibrosis. The symptoms and signs presented within one week after birth, with abdominal distension being the most common manifestation (90%). Radiographic evidence of intraperitoneal calcified plaques or masses were present in only half of our patients. Surgery was performed in eight, of whom four had primary bowel obstructions. The most common site of bowel perforation was at the terminal ileum (six patients). Two patients who did not receive surgical intervention were diagnosed radiographically and sonographically, and showed cystic type meconium peritonitis. The outcome of these patients has been good except for one who died of sepsis. CONCLUSIONS: The low mortality rate (10%) in this series may depend in part on successful prenatal diagnosis of four patients and, in part, on continuing progress in perinatal intensive care. A better outcome in this potentially fatal disease can thus be anticipated by the cooperation of neonatologists, pediatric surgeons and obstetricians.

Female↗

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↗

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↗

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↗

Factor VII deficiency with intracranial hemorrhage: a case report.

Factor VII deficiency is an autosomal recessive hereditary disorder characterized by a normal partial thromboplastin time and a prolonged prothrombin time. Definite diagnosis of this condition requires a specific Factor VII assay. Its quite uncommon occurrence in the newborn was first described by Alexander and associates in 1951. A one-day-old male, full-term newborn was presented here with skin pallor, respiratory distress and bitemporal bulging masses noted immediately after birth. Computed tomography scan of the brain revealed a subgaleal hematoma and subarachnoid hemorrhage. Laboratory studies revealed prolonged prothrombin time and a Factor VII level less than 1%. Literature on the clinical manifestation and management of Factor VII deficiency is reviewed briefly.

Cerebral Hemorrhage↗

Hyperglycemia induced by chemotherapeutic agents used in acute lymphoblastic leukemia: report of three cases.

Hyperglycemia may occur as a complication in patients with leukemia during induction therapy with L-asparaginase and steroids. The reported incidence is about 10%. The present report concerns three patients with acute lymphoblastic leukemia (ALL), complicated by hyperglycemia. Their ages were 10, 12, and 9 years, respectively. Past histories were normal, with no diabetes mellitus or other endocrine disorders in their families. Case 1 was an obese boy who developed pancreatitis and diabetic ketoacidosis (DKA) in his remission induction therapy which had included both L-asparaginase and steroids. Cases 2 and 3 both presented with polyuria and elevated postprandial blood sugar. For all patients, insulin was administered to control their blood sugars; the maximal daily dosage of insulin dispensed was 2.1 U/kg, 0.5 U/kg, and 0.7 U/kg, respectively. Increased plasma insulin and C-peptide levels suggestive of insulin resistance were observed in Case 3. The outcome of hyperglycemia in these three patients was good. The symptoms of this complication may vary from mild glucose intolerance to severe, or even fatal, DKA. Thus, periodic determinations of urine glucose and postprandial blood sugar are important for early recognition to prevent further life-threatening consequences.

Adrenal Cortex Hormones↗

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↗

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↗

The efficacy of intranasal DDAVP therapy in children with nocturnal enuresis.

This prospective self-controlled study was designed to evaluate the safety and efficacy of Desmopressin (DDAVP) in the treatment of childhood primary nocturnal enuresis (PNE) and the diurnal variation of ADH secretion and urine excretion/concentration in these children. Twenty-three children (15M,8F), aged 5-16 years, who wet their beds at least 3 nights per week were enrolled in the study. After a four-week observation period, they were hospitalized for one day to monitor intake, output, renal sonography, plasma ADH, urine and serum osmolality. Intranasal DDAVP treatment at a dose of 15-30 micrograms at bedtime was started with a 4-week titration period followed by a 3 to 6-month full dose treatment period. Subsequently the dose was tapered off for one to two months, and the patients were followed for at least two months to observe any recurrence. The results showed no diurnal difference of ADH level in these children (p > 0.05); serum osmolality decreased slightly during sleep (p < 0.01); urine production decreased, and urine osmolality increased, during sleep. Seventeen children (81%) responded with a more than 50% reduction in frequency of enuresis: 11 were excellent responders, 6 were partial responders, while 4 failed. After completion of therapy, four (19%) remained dry and were considered cured; the rest had much less frequent recurrence. There were no subjective complaints other than mild local discomfort; laboratory test results remained normal. It was concluded that intranasal DDAVP is a safe and effective treatment for PNE which usually works promptly. Given the spontaneous annual remission rate of 14%, the cure rate of 19% in this study was not satisfactory.

Administration, Intranasal↗

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↗

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↗

Acute lobar nephronia: a case report.

Acute lobar nephronia (ALN), a term analogous to acute lobar pneumonia, refers to a renal mass caused by acute focal infection without liquefaction. An alternative term is acute focal bacterial nephritis (AFBN). Fever, flank pain or back pain are the most frequently encountered clinical characteristics. Imaging modalities used to establish a definite diagnosis of acute lobar nephronia included sonography, computed tomography and nuclear medicine. Uroradiographic findings in this condition can mimic a renal abscess or neoplasm. Further distinction between acute lobar nephronia and other renal masses is aided by the appropriate use of renal sonography and computerized tomography is the most effective and least costly method in diagnosis. Serial sonograms can be used to monitor response to antibiotic therapy. Clinical and uroradiographic characteristics of a six year-old female patient with acute lobar nephronia are presented, along with a review of literature.

Acute Disease↗