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Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 109 records · Page 6Linked to original sources

[Menkes' kinky hair disease: report of 2 cases].

Menkes' kinky hair disease (trichopoliodystrophy) is a rare inherited X-linked recessive disease with an incidence of about 1:35,000, and is rare reported previously in Taiwan. We present 2 cases with typical features including sparse, coarse and stubby, kinky hair, depigmented skin, pudgy face, arrow-shaped upper lip, hypotonia, Babinski signs bilaterally, profound psychomotor retardation with disability of head control or rolling over, and poorly controlled myoclonic jerks. Both were male infants with a family history of male relatives died in early childhood. Their hairs showed pili torti and trichorrhexis nodosa microscopically. Serum levels of copper were 14 ug/dl and 20 ug/dl. Ceruloplasmin levels were 10.4 mg/dl and less than 7 mg/dl. Their EEG showed abnormal generalized brain polyspike waves. Brain CT scan showed generalized brain atrophy, and chronic subdural hematoma in case 1. Bilateral urinary bladder diverticula and spurs over the distal ends of the femoral diaphysis were found in case 1. Normal urinary bladder was found in case 2 initially, then diverticula developed one year later. They are currently on anticonvulsants (Rivotril) therapy. Repeated attacks of respiratory infection, myoclonic seizure, hypotonia, and static neurologic developmental status are noted.

Humans↗

Brain abscesses in children--a cooperative study of 83 cases.

This study reviews our experience in 83 cases of brain abscesses in children diagnosed at seven teaching hospitals during the 10-year period from June 1978 to July 1987. The average age of the patients was 7 years, with 12% of them less than 1 year old. The male-to-female ratio was 1.7:1. Of the brain abscesses, 90.4% were detected by CT brain scan. A total of 50.6% patients had congenital heart disease, and 20.4% patients had sepsis and/or meningitis. Only 6% cases had ear, nose, and throat infection. Sixty-eight (81.9%) patients received a combination of antibiotics and surgical treatment. The cerebral abscesses were totally excised in 26 cases, aspirated and partially excised in 6, and aspirated in 32. Sixty patients had pus cultures from the cerebral abscesses. Organisms were isolated in 29 (49.2%) of them. Streptococcus was by far the most common organism. The overall outcome was: 49 (59%) alive; 16 (19.3%) dead; 18 (21.7%) lost to follow-up. Among the 16 mortalities, the causes of death were due to failure to treat the diseases causing the brain abscesses. We had a better outcome in patients whose cerebral abscesses were totally excised or whose abscesses were aspirated, and in patients who were older than 1 year of age.

Adolescent↗

An acute pleuropneumonia in a pig caused by Chromobacterium violaceum.

A 2.5-month-old, 30 kg Duroc pig died 10 days after showing clinical signs of dyspnoea and diarrhoea. Acute necrotizing and fibrinous pleuropneumonia with locally extensive lesions was found. Chromobacterium violaceum was isolated from pneumonic lung tissues and intratracheal inoculation of a pure culture into two SPF pigs reproduced lesions similar to those found in the natural infection.

Acute Disease↗

Improvement of modern treatment and outcome in childhood epilepsy in Asia.

Epilepsy and epileptic syndromes are one of the major pediatric neurological diseases in Taiwan, R.O.C. In 1984 we investigated 38 elementary schools in Taichung city, in the middle-west part of Taiwan. Among 57,944 school-aged children, 388 had suffered from at least two episodes of afebrile seizures occurring separately over a two-week period. The period prevalence rate was 0.67%. In our clinic, once seizures are diagnosed, the seizure types are then classified as well as possible, in order to develop a rational approach to treatment. We commonly use carbamazepine for partial seizures and some generalized seizures, low dose ACTH (10 I.U.) for infantile spasms, valproic acid for absence seizures and myoclonic seizures, clonazepam for atonic-akinetic and myoclonic seizures, and phenobarbital for young children with generalized seizures. In the last three years, we have used a ketogenic diet for akinetic-atonic seizures and Lennox-Gastaut syndrome; we have also used temporal lobectomy for complex partial seizures since 1981. Owing to the marked increase in the medical understanding of epileptic seizures and syndromes, and models of treatment, there is no question that children with seizure disorders are better off today than they were ten years ago.

Adrenocorticotropic Hormone↗

[Malignant fibrous histiocytoma of spleen. A case report and literature review].

A 11 year old girl who presented with malaise, poor appetite and flank mass was admitted in Sept. 1988. Abdominal x-ray, sonogram, Gallium scan and CT scan revealed a tumor mass in the spleen. Laparotomy confirmed a hugh mass measuring 15x15x10 cm in the spleen. The histologic study proved the tumor to be malignant fibrous histiocytoma, inflammatory type. Malignant fibrous histiocytoma occur mainly in late adult life, we report this case because the patient is young and the involvement of the spleen is rare.

Child↗

[Childhood hypoplastic preleukemia].

Aplastic anemia preceeding acute leukemia is known as hypoplastic preleukemia, which tends to be more frequently evolved into acute lymphoblastic leukemia, whereas myelodysplastic preleukemia are specific for acute myelocytic leukemia. More than forty cases had been reported in the literatures. Here we report a 11-year-old boy who was initially diagnosed as aplastic anemia and treated with prednisolone and androgen with prompt response terminated into overt acute lymphoblastic leukemia.

Anemia, Aplastic↗

[Acute peritoneal dialysis in low birth weight infants].

Between September 1986 and April 1988, five low birth weight infants weighing 950 gm to 2250 gm required acute peritoneal dialysis due to acute anuric renal failure. Severe hyaline membrane disease was the most common explanation for acute renal failure. All infants had severe electrolytes imbalance and fluid retention with generalized edema, which increased ventilation requirements and severely limited fluid intake. The volume of each exchange varied between 10-20 ml/kg. Four of five infants had good ultrafiltration (3.52 +/- 2.43 ml/kg/hr, mean +/- SD). Ultrafiltration was not achieved in one due to poor peripheral perfusion. Electrolytes imbalance were corrected in all patients. Although four infants died, one case given up further care after renal failure had improved, and one died of septic shock after renal function had recovered. Only one infant survived with complete recovery of renal function. We believe that early dialysis is likely to reduce both morbidity and mortality of acute renal failure in low birth weight infants.

Acute Kidney Injury↗

[Urinary tract infection in children: clinical analysis and investigation].

The urinary tract is a common site for bacterial infection in childhood. The most important aspect of urinary tract infection in childhood is that it can lead to severe and progressive renal disease. From March 1983 to March 1987, 63 cases of suspected urinary tract infection were collected in Taichung VGH. Among them only 24 cases reached our diagnostic criteria. Eight cases were within 2 years old, which all are male. Sixteen cases were beyond 2 years old, which the ratio of male to female is 1:1. Eighteen cases presented pyuria. Ten cases had recurrent infections, among which eight cases had urinary abnormalities. The most common pathogenic organism was E. coli (44%), then Proteus (24%), Klebsiella (16%), Pseudomonas (12%). The antibiotics sensitivity test revealed 95.7% were sensitive to gentamicin and amikacin, 81% to cephalothin, 56. 5% to sulfamethoxazole-trimethoprim. Imaging studies revealed nine cases (37.5%) with urinary tract abnormalities, including vesicoureteral refluxes 6 cases, right multicystic kidney with left vesicoureteral reflux 1 case, horse-shoe kidney 1 case, atonic bladder 1 case. The ratio of male to female is 2:1. All seven cases of severe vesicoureteral refluxes received surgery. One case developed post-operative ureteral stricture. One case occurred unilateral chronic renal parenchymal disease one year later. Others were free of reflux and reinfection of urinary tract.

Anti-Bacterial Agents↗

Aicardi syndrome--a case report.

The Aicardi syndrome was first described by Aicardi in in 1965, which consisted of infantile spasms, defect of the corpus callosum, a characteristic lacunar chorioretinopathy, mental subnormality, and costovertebral anomalies. All patients have been female except one male case was reported in Australia. There have been approximately over 100 cases of the Aicardi syndrome reported in the literatures since 1965. The etiology of this syndrome is unknown. The most likely cause, however, is an X-linked mutational event with lethality in hemizygous male. We report a 43-day-old female infant with classic features of Aicardi syndrome including flexion spasms, dysgenesis of corpus callosum, microphthalmia, characteristic EEG, and characteristic lacunar chorioretinopathy.

Agenesis of Corpus Callosum↗

[Cerebrospinal fluid eosinophilia preceding central nervous system leukemia].

Cerebrospinal fluid eosinophilia are an uncommon finding that is most often the result of a helminthic infection of the central nervous system. Information from the recorded literature suggests the differential diagnosis of this clinical observation is relatively limited. This is a report of a girl with acute lymphoblastic leukemia who developed central nervous system relapse of leukemia following 3 years of remission. She showed marked eosinophilia in the cerebrospinal fluid 6 weeks preceding central nervous system leukemia. No cause for the eosinophilia was identified. The gradual accumulation of case reports of eosinophilia in the cerebrospinal fluid with or without blood eosinophilia in patient with leukemia suggests that this finding is of some importance.

Brain Neoplasms↗

[Clinical observation of neonatal sepsis].

Sepsis remains a significant cause of morbidity and mortality in newborn infants. From January 1983 to April 1988, 166 cases of neonatal sepsis with positive blood cultures were collected at V.G.H.--Taichung. Among them 140 newborn babies were delivered at private clinic (outborn babies), 26 cases were inborn babies. Of the inborn babies, 20 cases (76.9%) were early onset sepsis (the onset of illness within 96 hours of life) and 6 cases (23.1%) were late onset sepsis (the onset of illness beyond 96 hours of life). Off the outborn babies, 64 cases (45.7%) were early onset sepsis and 76 cases (54.3%) were late onset sepsis. The Gram positive organism (51.9%) was more common than the Gram negative organism in the inborn babies, on contrary, the Gram negative organism (59.0%) was more common in the outborn babies. The most common pathogenic organism of the inborn babies was Enterococcus (22.2%) and E. coli (22.2%), followed by Pseudomonas spp (11.1%) and Staphylococcus aureus (11.1%). The most common pathogenic organism of the outborn babies was Enterococcus (17.4%), followed by E. coli (16.1%), Staphylococcus aureus (9.9%) and Klebsiella spp (8.1%). The antibiotics sensitivity tests to the pathogens didn't show any significant difference between these two group babies. In this clinical study, we found that the first choices of antibiotics were ampicillin plus aminoglycosides. The clinical symptoms and signs were nonspecific. The most common findings were lethargy, fever, hypothermia and poor feeding. Of the inborn babies, 17 cases (65.4%) had the predisposing factor(s). Of the outborn babies, 42 cases (30%) had the predisposing factor(s).(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗

Anomalous origin of one pulmonary artery from the innominate artery: a report of two cases.

Two children with an anomalous origin of one pulmonary artery from the innominate artery are reported. One was a 15-month-old boy presenting with respiratory distress. He had a right aortic arch and his left pulmonary artery originated from the innominate artery. The other was a 1-month-old girl presenting with congestive heart failure. She had a left aortic arch and her right pulmonary artery originated from the innominate artery. An understanding of the embryological pathogenesis of these anomalies has significant therapeutic implications.

Brachiocephalic Trunk↗

Intracardiac ultrasound assessment of atrial septal defect: comparison with transthoracic echocardiographic, angiocardiographic, and balloon-sizing measurements.

PURPOSE: Accurate evaluation of the size, location and adjacent structure of an atrial septal defect (ASD) is very important in the selection of patients for further management. We directly compared the utility of transthoracic echocardiography, angiocardiography, balloon sizing, and intracardiac ultrasound (ICUS) in the detection of ASD. METHODS: Twenty-one children underwent an ICUS study of ASD after routine clinical and laboratory studies. All patients had received transthoracic echocardiography (TTE), cardiac catheterization, cineangiography, and balloon sizing before the ICUS to evaluate the ASD. RESULTS: There was a significant correlation between the ICUS-derived ASD diameter and the other methods (p < 0.001). The balloon-sizing diameter was estimated by the equation: TTE diameter x 1.09 + 3.9 mm. There was a good correlation between the predicted and measured balloon-sizing diameter (r = 0.963; p < 0.001). CONCLUSION: It is worthwhile spending a few minutes to perform ICUS during cardiac catheterization since it will provide more detailed information on and high resolution images of atrial septal morphology, especially for patients undergoing transcatheter closure by device.

Adolescent↗