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Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 91 records · Page 5Linked to original sources
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

Adult respiratory distress syndrome in children.

Adult respiratory distress syndrome or ARDS as coined by Ashbaugh et al in 1967, has been a great challenge in the field of critical care since then. It is a clinical entity which can be caused by various insults at any age. There have been several case reports of ARDS involving infants and children in the past 10 years, but pediatric ARDS is still not well recognized in Taiwan. A review of admissions to the pediatric intensive care unit in the past 2 years shows that 11 of the cases were included as pediatric ARDS combined with the expanded definition of Murray et al, and that each patient had an acute lung injury score greater than 2.5. Clinical manifestations also presented acute pulmonary distress indicating ARDS. The distribution of age ranged from 13 months to 11 years. The predisposing insults included sepsis, gastrointestinal bleeding with shock and massive blood transfusion, central nervous system infection, major trauma, near drowning, fulminant hepatitis and chemotherapy for acute leukemia. All received mechanical ventilatory support. The average peak inspiratory pressure was 46.7 +/- 6.4 cmH2O and the mean value of maximal PEEP used was 11.9 +/- 4.4 cmH2 O. Three patients developed barotrauma. Two patients survived and nine expired, a mortality rate of 82%. It is important for physicians caring for infants and children with respiratory failure to consider the diagnosis and initiate adequate ventilatory support and other resuscitation management.

Child↗
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↗
PubMed1993

[Cantrell's pentalogy: a case report].

Cantrell's pentalogy is a congenital anomaly resulting from embryologic development defect. The anomalies observed in this disorder were: (1) a midline, supraumbilical abdominal wall defect, (2) a defect of the lower sternum, (3) a deficiency of the anterior diaphragm, (4) a defect in the diaphragmatic pericardium, (5) congenital intracardiac defects. Its occurrence in the newborn is quite uncommon, though it was first described by Cantrell and his colleagues in 1958. A one-day-old male fullterm newborn was presented. Omphalocele and partial ectopia cordis were noted immediately after birth. Echocardiogram revealed tetralogy of Fallot with atrium septal defect. Operative findings were sternal cleft, pericardial defect and ventral diaphragmatic defect. The literature on clinical manifestations, embryogenic defect and management of Cantrell's pentalogy is reviewed briefly.

Abdominal Muscles↗
PubMed1992

MRI of sequela of transverse myelitis.

A 4-year-old boy developed acute paraplegia, associated with sensory impairement and bowel and urinary dysfunction after an URI. MRI showed diffuse hyperintensity in T2WI in the spinal cord below the T6 level. Acute transverse myelitis was diagnosed based on the clinical presentations and MRI findings. The patient had poor recovery and two months later, a follow-up MRI disclosed a severe diffuse atrophic change of the spinal cord in the affected segment.

Acute Disease↗
PubMed1992

Roentgenographic determination of total lung capacity in normal Chinese children.

Roentgenographic determination of total lung capacity (TLC) in children, rarely mentioned in the past, differs from the determination of TLC using helium dilution method in that it does not need the measurement of functional residual capacity. Therefore, it is useful for hospitals where pulmonary function testing (PFT) is not available. In this study, 87 out of 125 normal children completed both chest radiographs and PFT. Ellipsoid method which divides the lung field into several sections was used to calculate TLC from chest radiographs. The correlation coefficient of TLC measured by both chest radiographs and PFT was 0.7680, and paired Student's t-test of the mean values of the two groups showed P value greater than 0.05. Among the variables of height, weight, age and body surface area, height alone was able to predict the normal reference value of TLC. Line of regression between TLC and height was expressed as "TLC = 52.776 x Height- 4404.998", and correlation coefficient was 0.8013. Intersubject reproducibility was also verified. It is concluded that chest radiographs can be used to determine TLC in children.

Adolescent↗
PubMed1992

[Double aortic arch-three cases report and operative treatment].

This report is on three double aortic arch cases. They were diagnosed in our department between 1982 and 1992. The first case was complicated by dilated cardiomyopathy whose diagnosis was delayed due to a lack of clinical experience. Corrective surgery relieved the airway obstruction and his breathing improved postoperatively. The patient finally died of heart failure due to concurrent progressive dilated cardiomyopathy one and half years later. The second and third cases were diagnosed on the day of admission by a barium esophagogram and echocardiogram. MRI (Magnetic resonance image) of the cardiovascular system on these two patients revealed no other associated abnormalities. No angiography was done on the third case. They underwent surgery with excellent results. In any infant younger than 3 months with dyspnea and dysphagia, double aortic arch should be suspected. The esophagogram can show extrinsic compression. An echocardiogram can reveal two aortic arches. Both procedures can be performed easily and safely at the bedside. We recommend that these to be considered as routine examinations in such patients.

Aorta, Thoracic↗
PubMed1991

[Cyclosporin A for treatment in nephrotic syndrome of childhood--a preliminary report].

We treated five, three steroid resistant and 2 steroid dependent nephrotic syndrome with oral cyclosporin A and prednisolone. All children received more than eight weeks course of prednisolone, and were in a critically ill status from their nephrotic syndrome and by steroid-toxic side effects. Cyclosporin A was started at 7 mg/kg/day and titrated to maintain serum level of 150-250 ng/ml. Prednisolone was given initially at a dose of 1 mg/kg/day x 1 month, subsequently at a dose of 0.4 mg/kg/day x 1 month, then 0.2 mg/kg/day x 4 months. After a total course of six months, cyclosporin A was tapered to 3.5 mg/kg/day. Prednisolone was maintained at a dose of 0.2 mg/kg/day. Renal biopsies of the 3 steroid resistant children showed membranous glomerulonephritis with focal segmental glomerulosclerosis, focal segmental glomerulosclerosis and IgM nephropathy respectively. These three cases all went into complete remission within four weeks. Among them, one case relapsed twice within one year. Renal biopsies of the two steroid dependent children showed minimal change and focal segmental glomerulosclerosis respectively. These two cases went into complete remission within three weeks sustained up to a year. Side effects of cyclosporin A were observed in one patient with gum hypertrophy and two patients with hirsutism. Other side effects were not found. After cyclosporin A treatment for one year, the renal biopsy of the relapsing patient showed no interstitial fibrosis or tubular atrophy. In conclusion, cyclosporin A was found to be effective in steroid resistant and dependent patients. The duration of cyclosporin A treatment is so far unknown. It needs further evaluation in the future.

Child↗
PubMed1991

[Nemaline myopathy--a case report].

An 8-year-old Chinese girl who was noted to have feeding difficulty and poor weight gain since her infancy. Her motor development was delayed with sitting at 1-year-old and walking independently at 2-year-old. She visited our OPD when she was 8. Physically, she was thin and slender. Her height and weight were below the 10th and 3rd percentile. Her face was elongated with high arched palate. Neurologically, she had waddling gate with marked proximal muscle weakness. Her cranial nerves and sensation remained intact. Laboratory tests including blood routine, liver function, renal function, electrolytes, NCV and cardiac echo all were within normal limits. The EMG showed myopathic pattern. A fresh-frozen muscle biopsy stained with the modified Gomori trichrome, processed by histochemistry and electronmicroscopic examination showed typical nemaline rods with predominance of type 1 muscle fibers. She was then diagnosed to have nemaline myopathy.

Child↗
PubMed1991

[Lennox-Gastaut syndrome with band form heterotopia: a case report].

A 10-year-old girl with developmental delay, mental retardation and intractable seizures is reported. She manifested clinically as Lennox-Gastaut syndrome with unknown etiology before admission. Brain MRI revealed a band-like lesion over the bilateral subcortical region being later recognized as band-form heterotopia. This type of heterotopia may be overlooked easily due to its symmetrical distribution and mild degree of cortical convolutional anomaly. As it is often associated with intractable seizures and psychomotor retardation, the prognosis is poor. This patient responded poor to ketogenic diet and anticonvulsants. We present this case and review the related articles.

Brain Neoplasms↗
PubMed1991

[Double aortic arch associated with severe left ventricular dysfunction--a case report].

A case of a 3-month-old male infant with severe LV dysfunction and a complete double aortic arch (both arches patient) is reported. The right dominant aortic arch runs retroesophageally to the right and joins with the left smaller arch to form the descending thoracic aorta on the right side while the ligamentum arteriosum was located in its normal position (the left side). Through a left thoracotomy, the smaller left arch and the ligamentum were divided to relieve the obstruction of the trachea from compression of the vascular ring. Postoperative convalescence was good. Further follow up of this patient after 9 months showed that there was neither any respiratory failure nor any heart failure sign present, though severe LV dysfunction persisted.

Aorta, Thoracic↗
PubMed1991

Mitochondrial encephalomyopathy presenting with clinical Leigh's disease: report of a case.

Leigh's disease is one of the mitochondrial encephalomyopathies. This article presents a 7-month-old baby boy who had been well-being since birth until 6 months of age when episodic downward gaze of both eyes with limitation of horizontal eye movement were noted. This episode of cranial nerve palsies lasted about 4-5 days and subsided spontaneously. The second attack was noted one month later, to be associated with hypotonia and truncal ataxia. Episodic hyperventilation with resultant gasping and myoclonus was noted at the third attack but spontaneous respiration resumed soon with persistent ophthalmoplegia and truncal ataxia. Lumbar puncture, brain MRI, amino acid assay and cardiac echo all showed negative finding. The oral glucose lactate stimulation test revealed an elevation of lactic acid, brain stem evoked potential indicated bilateral obscure 4th and 5th waves, and muscle biopsy showed ragged red fibres with aggregation of structurally abnormal mitochondria noted under electron microscope. Coenzyme Q, thiamine and carnitine had been given before biochemical study; however, the neurological symptoms did not show any improvement. Biochemical study finally revealed normal respiratory chain enzymes including NADH-coenzyme Q reductase, succinate coenzyme Q reductase and cytochrome c oxidase while other enzymes were technically unavailable for study. Unfortunately the patient died at 18-month-old due to respiratory failure.

Humans↗
PubMed1990

Continuous monitoring of intracranial pressure in Reye's syndrome--5 years experience.

Monitoring of intracranial pressure (ICP) and efforts to keep the ICP below the critical level are vital in the treatment of Reye's syndrome. Continuous monitoring of ICP was carried out in 21 cases of Reye's syndrome who were at or beyond stage III at the time of admission to the Veterans General Hospital, between January 1981 and August 1986. Seventeen had ICP ranging from 15 mmHg to 67 mmHg. Three patients died, 1 in stage V with an ICP of 67 mmHg received a craniectomy, and 2 others were in stage IV with ICP's of 66 mmHg and 25 mmHg, respectively. The fatality rate was 14% (3/21). Among 18 patients, 5 had moderate psychomotor retardation (PMR), 4 had severe PMR and 2 had mild PMR. The remaining 7 patients survived without sequelae. Blood exchange transfusion could further reduce ICP and seemed to improve neurologic outcome. Blood ammonia higher than 400 micrograms% is indicative of a bad prognosis. Hyperventilation was the most rapid and effective means of reducing moderate degrees of increased ICP. During intensive supportive care, we also found that coughing, endotracheal intubation, seizures, asynchronous respiration to an artificial respirator, suction of the airway and any painful stimulation caused further increases in ICP and worsened the situation. Care should be given to avoid these factors.

Ammonia↗
PubMed1990

[Retroperitoneal fibrosis in children].

A 13 year-old boy was hospitalized because of persisted fever had pallor for three months. Abdominal ultrasound, intravenous pyelogram, and abdominal computerized axial tomography scan disclosed a space occupying lesion located postero-superior to the bladder. Biopsies of the above lesion disclosed retroperitoneal fibrosis. Retroperitoneal fibrosis in children is very rare and has a wide variety of presentations and associated diseases. Treatment relief of obstruction when necessary.

Humans↗
PubMed1990

Arnold-Chiari type 1 malformation and syringomyelia.

A 12-year-old boy had a long history of headache since the age of 4 years. Numbness and weakness of the upper extremities and chest tightness, especially in the morning, were noted since 2 years ago. The headache became more severe and frequent and attacked even in sleep since one year ago. Arnold-Chiari type 1 malformation, cervical syringomyelia and mild hydrocephalus were demonstrated by MRI scan of the central nervous system. Headache and other symptoms disappeared immediately after surgical intervention. The formation of syringomyelia may be multifactorial, but Arnold-Chiari type 1 malformation and uneventful birth history play an important role in the underlying cause of this case. We presented this youngest case who had Arnold-Chiari type 1 malformation and onset of headache from the age of 4 years, discussed the pathogenesis of syringomyelia and reviewed the literature.

Arnold-Chiari Malformation↗