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Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 145 records · Page 8Linked to original sources
PubMedDate not supplied

Graves' disease associated with myasthenia gravis: report of one case.

Exophthalmos occurs in more than half of the patients with childhood Graves' disease, while severe Graves' ophthalmopathy is uncommon in children. Myasthenia gravis is even more rare in association with Graves' disease. A 7-year-old girl is reported; she had ptosis for two years; this was treated irregularly, and she then developed exophthalmos, ophthalmoplegia and thyroid goiter. Her laboratory tests showed T3 496 ng/dl, T4 17.05 ug/dl, TSH < 0.1 uU/ml, fT4 > 4.2 ng/dl. Antithyroglobulin antibody was 1:25600 and antimicrosomal antibody was 1:102400. Myasthenia gravis was confirmed by a positive neostigmine test. Due to allergic reaction to propylthiouracil, she was treated with carbimazole and propranolol. Thereafter the thyrotoxicosis was was well controlled. The ocular-type myasthenia gravis remained stable with minimum medical treatment.

Child↗
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↗
PubMedDate not supplied

[Non-surgical treatment of spinal epidural abscesses: report of one case].

Spinal epidural abscesses (SEA) are uncommon in children. This paper reported a two-year-old boy who was noted to have neck stiffness, with local tenderness posteriorly. Bacterial meningitis was suspected initially in terms of the finding of the cerebral spinal fluid; antibiotics were prescribed. Three days later another spinal tap was performed because of persistent high fever and irritability. A pus-like material drained out as the needle punctured into the spinal region. A magnetic resonance image (MRI) scan of the spine revealed a SEA, with extensive involvement from the second cervical spine to the lumbosacral spine region. Culture of the pus, as well as the blood and CSF, were positive for Staphylococcus aureus. Because of extensive involvement of the spinal epidural space, the patient was again given antibiotics: Prostaphllin and Amikin intravenously for six weeks instead of laminectomy. Then the oral antibiotic (Keflex) was given to the patient for another three months after the boy was discharged from the hospital. A review of the literature shows the incidence of SEA to be increasing and the bacterial spectra to be broadening because of increasing use of immunosuppressing drugs or antibiotics, and the increase in numbers of immunecompromised patient. The clinical symptoms and signs of the SEA were non-specific, but SEA can be early diagnosed by computurized tomography (CT) scan or MRI scan with caution. The literature suggests that, if the patient's condition fits the criteria for non-surgical treatment, antibiotic therapy is the first choice for preventing the complication of spinal deformity, especially in children.

Abscess↗
PubMedDate not supplied

Cerebral magnetic resonance imaging of preterm infants after corrected age of one year.

A total of 17 very-low-birth-weight (range from 625-1500 grams) preterm infants received brain magnetic resonance imaging (MRI) study at a mean corrected age of 23 months (range from 15-33 months). They were classified into two groups. In Group 1 (12 cases) the cranial ultrasound study was normal during infancy. There were two cases with typical change of periventricular leukomalacia (PVL) detected by MRI study; both showed spastic diplegia. In Group 2 (five cases) ultrasound study was abnormal during infancy while the MRI confirmed the change of PVL in these cases whose ultrasound study showed typical change of PVL. In two cases of intraventricular hemorrhage (IVH) Grade I and III separately, the MRI also revealed the change of PVL, but only the case of IVH grade III had spastic diplegia. The myelination process of all cases were completed as normal term infants. In conclusion, brain MRI confirmed the PVL change discovered by cranial ultrasound and the results were strongly correlated with spastic type cerebral palsy. For those infants with an abnormal neurological examination, MRI may be useful for diagnosis of PVL in spite of normal ultrasound findings during the newborn period.

Brain↗
PubMedDate not supplied

Lesch-Nyhan Syndrome: report on two brothers.

Lesch-Nyhan syndrome is a rare X-linked disease characterized by over-production of uric acid and a central nervous system (CNS) disorder consisting of mental retardation, spasticity, choreoathetosis, and a compulsive form of self-mutilation. A deficiency in hypoxanthine-guanine phosphoribosyl transferase (HPRT) provides the underlying metabolic basis for this disease. A 12 month-old male baby who had orange crystals over the diapers since he was 3 months old was brought to our hospital due to developmental delay. Mental retardation and athetosis were also noted. Chemical analysis revealed hyperuricemia (uric acid 8.6 mg/dl). Urine routine showed microscopic hematuria and uric acid crystals. The activity of HPRT in erythrocyte lysates of parents were both within normal limits, but that of the patient was very low (0.0547 nm/min/mg protein, < 0.05% of control). His younger brother was born 2 months after this disorder diagnosed in this patient. The younger brother was noted to have uric acid crystals over the diapers when he was 40 days old and hyperuricemia (10.6 mg/dl) showed up later. He was also a case of Lesch-Nyhan syndrome since the activity of HPRT in erythrocyte lysates was also low (0.0327 nmol/min/mg protein, < 0.05% of control). Further studies, including carrier detection and deoxyribonucleic acid (DNA) analysis, could be helpful for genetic counseling. This syndrome is rare among Chinese, and this may be due to underdiagnosis.

Family Health↗
PubMedDate not supplied

Langerhans cell histiocytosis: a 10-year review.

Hospital records of seventeen patients (11 males & 6 females) with Langerhans cell histiocytosis, confirmed by electron microscopic demonstration of Birbeck granules, were studied retrospectively from October 1982 to October 1992 at Taichung Veterans General Hospital. The ages at presentation ranged from 5 months to 17 years (a median of 6 years). The clinical features were protean and included fever, pain, bony lesions, lung lesions, abnormal dentition, diabetes insipidus, oral ulcer, otorrhea, dermatitis, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. Skull and femur were the most common sites of bony lesions. The main therapeutic modalities were excision, radiotherapy, and chemotherapy with vincristine and prednisolone. The young age at presentation, several involved organ systems, presence of organ dysfunction, and clinical diagnosis with Letterer-Siwe disease were poor prognostic factors. Although Langerhans cell histiocytosis is not a rare disease, the cause is still unknown. It needs further research to disclose the mystery.

Adolescent↗
PubMedDate not supplied

Clinical and MRI study of the Hallervorden-Spatz syndrome: long-term follow-up of one case.

Hallervorden-Spatz syndrome is generally considered to be an autosomal recessively hereditary disorder of unknown etiology. Some reported cases have been known to be sporadic. We present a boy who suffered from regressive developmental milestones since he was 2 years and 6 months old. He began to manifest tremors of the upper extremities, followed by unsteady gait, choreoathetosis, dystonia, dysarthria, and dysphagia at 4 years old, and subsequently became completely bedridden at 6 years old. Neurologically, opisthotonus, rigidity of extremities, dystonia, hyperreflexia, profound emaciation, and bilaterally positive Babinski signs were present. The brain magnetic resonance imaging (MRI) done at the age of 8 years revealed symmetrical low signal intensity over the bilateral globus pallidi in the T2-weighted images coexistent with an area of high signal intensity over the anteromedial portion, known as "eye of the tiger" sign. Another MRI, followed up two years later, did not show marked difference in signal abnormalities over the globus pallidi in the T2-weighted images as compared with that of the previous one. However, progressive neurological deterioration existed.

Brain↗
PubMedDate not supplied

Lipomyelomeningocele: a 9-year review.

Fourteen infants and children, 6 males & 8 females, with lipomyelomeningoceles were reviewed from October 1982 to December 1991 at Taichung Veterans General Hospital. The age at diagnosis ranged from 3 days to 5 years. The chief problems included mass on the back, urinary distention, weakness of lower limbs and poor bowel control. The cutaneous lesions over the lumbosacral region were subcutaneous lipoma, dimples and hemangioma. There were several associated anomalies, such as occult spina bifida, syringomyelia, sacrococcygeal dysgenesis, high-types imperforate anus, genitourinary anomalies, congenital heart disease, talipes equinovarus and annular pancreas. Among the 12 cases who were operated on, 8 had preoperative neurological deficits, but there was only little postoperative improvement in 2. It is important to recognize at an early stage the defect underlying skin lesions and its associated anomalies, rather than to be concerned about cosmetic factors. Early surgical repair before the appearance of neurological deficits is recommended.

Child, Preschool↗
PubMedDate not supplied

Benign partial epilepsy with centrotemporal spikes: analysis of 94 Chinese children.

From October 1982 to June 1992, 94 neurologically normal Chinese Children (50 males & 44 females) with benign partial epilepsy of childhood with centrotemporal spikes were studied among 1847 epileptic infants and children. Their ages at onset were between 3.6 and 12 years (a mean of 7.3 years). All of them had normal birth and developmental histories. Seven patients (7.5%) had past history of febrile convulsion and 12 cases (12.8%) had family history of epilepsy. The depicted seizure patterns were protean, but most attacks (83%) occurred during sleep (nocturnal & diurnal). The duration of active period and seizure frequency had no significant difference in terms of sex and treatment, but early-onset patients (< 7.3 years) had statistically significant longer duration of active period than that of late-onset group (p < 0.01). We recommend that antiepileptic drug treatment is not essential for this disease. Even if antiepileptic drug is used, the low-dosed carbamazepine is suggested.

Carbamazepine↗
PubMedDate not supplied

Evolution of childhood absence epilepsy, juvenile myoclonic epilepsy and epilepsy with grand mal on awakening.

Seventy-one Chinese children with idiopathic generalized epilepsy, from among 1931 pediatric epileptics, were reviewed clinically and electroencephalographically. They were classified under childhood absence epilepsy (CAE, 50/71), juvenile myoclonic epilepsy (JME, 7/71) and epilepsy with grand mal on awakening (GMA, 14/71). All the patients' family members with epilepsy were classified under idiopathic generalized epilepsy. Hyperventilation was the most common precipitating factor for CAE, and sleep deprivation was the most common one for JME and GMA. Electroencephalographically, fast generalized spike or polyspike-wave complex (> or = 3Hz) was the most common feature. Three JME (42%) and 4 GMA patients (28%) had been diagnosed as CAE about 5 to 7 years preceding JME and GMA, respectively. Sixteen CAE cases (32%) later evolved to be GMA. All JME patients had concomitant GMA and three GMA cases (21%) had concomitant JME at diagnosis. There is strong evolutional correlation among CAE, JME and GMA in this series.

Adolescent↗
PubMedDate not supplied

Clinical manifestation of mitochondrial diseases in children.

Fourteen patients (10 boys, 4 girls) aged from 4 months to 14 years old were diagnosed with mitochondrial disease based on the clinical manifestations together with abnormal muscle mitochondrial morphologies. Their clinical diagnoses included Leigh syndrome, three; Menkes' syndrome, three; Kearns-Sayre syndrome, two; myoclonic epilepsy with ragged fibres, one; and infant-onset progressive myoclonic epilepsy, one; fatal infantile mitochondrial myopathy, one; fatty acid oxidation defect, two; and myopathy with cardiopathy, one. Organs involved other than muscles included central nervous system, ten; heart, six; eye, two; liver, two; and kidney, two. Clinical manifestations varied to include hypotonia, seizures, myoclonus, mental retardation, nystagmus, ataxia, ptosis, ophthalmoplegia, retinal degeneration, muscle atrophy, spasticity etc. Nine had an abnormal rise in lactate after glucose loading. Ragged-red fibres were found in four patients. Abnormal mitochondrial morphology included abnormal accumulation, abnormal cristae pattern of tubular, concentric, or parallel form, some contained osmiophilic inclusion bodies. One patient of Leigh syndrome had had brain necropsy which showed intramyelin splitting of myelinated axons.

Adolescent↗
PubMedDate not supplied

Intramyelin splitting in the spongiform lesions of Leigh syndrome.

A 5-month-old male infant, diagnosed as Leigh syndrome, presented with developmental delay, episodic apnea, metabolic acidosis, and myoclonic seizures. The magnetic resonance image of brain showed multiple symmetric low signals over the putamen in the T1-weighted axial view and linear high signal over the brainstem in the T2-weighted sagittal view. On electron microscopy, subsarcolemmal aggregation of abnormal mitochondria in muscle cells were found via biopsy. Small samples of myocardium, liver and brainstem, obtained via necropsy, were subjected to pathological examinations. On light microscopy, vacuoles were found in the myocardium and hepatocytes, while spongiform lesions in the neuropils, round cell infiltration, and vascular proliferation were observed in the brainstem. On electron microscopy, intramyelin splittings were observed in the spongiform lesions. We believe that intramyelin splitting must play an important role in the pathogenesis of spongiform lesions in Leigh syndrome.

Humans↗
PubMedDate not supplied

Cystic fibrosis in two Chinese infants in Taiwan.

Cystic fibrosis was diagnosed in two female infants, respectively nine months old (Case 1) and six months old (Case 2). Clinical presentation of these two infants showed frequent episodes of pneumonia, dehydration and poor weight gain, despite fair intake of food since birth. Hyponatremic, hypochloremic metabolic alkalosis had been found during their previous hospitalizations. In Case 1, stool fat was positive with negative trypsin test. Both cases had abnormal findings on chest X-ray films, but the pancreas showed no remarkable cystic changes from sonography. Plastic-bag method of sweat test showed Na/Cl: 155/185 nmol/L in Case 1, Na/Cl: 127/135 nmol/L in Case 2. A family history was suggested, since each one had an elder brother who had died in early infancy with similar clinical presentations. Both patients were put on special diet therapy as well as chest physical therapy. Case 1 died suddenly at 16 months of age. Autopsy revealed mucusplugged dilated ducts with atrophy of the exocrine portion of the pancreas. DNA analysis in Case 2 showed abnormal mutation point at 1898+5 G-->T on chromosome 7.

Cystic Fibrosis↗
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Intraventricular hemorrhage in preterm neonates--a two year experience.

A prospective brain sonograph study was performed on 134 preterm neonates with birth weight of less than 2500 gm. Ten (7.5%) infants had intraventricular hemorrhage (IVH). The incidence of IVH in preterm neonates weighing less than 1500 gm was 13% and in neonates of less than 32 weeks gestation was 11.27%. Ninety percent of the IVH occurred within the first 72 hours of life. Prematurity, and hypoxia were considered as significant contributing factors for neonatal IVH. Six of the 7 infants with IVH grade I have a favorable neurological outcome. One of the 3 infants with IVH grade III has psychomotor delay. Routine brain sonograph study is considered practical and invaluable for diagnosis of IVH in preterm neonates.

Cerebral Hemorrhage↗
PubMedDate not supplied

Candidemia in the neonatal intensive care unit.

The hospital records of 18 infants (9 males & 9 females) with one or more positive cultures for Candida species were studied retrospectively in an attempt to define the characteristics, associated factors and treatment for candidemia in the neonatal intensive care unit. The number of patients have increased recently and the mortality rate is 56% (10/18). The Candida species isolated from blood were Candida albicans in 16 cases and Candida parapsilosis in 2 cases. Fever, not-doing-well, and abdominal distention were the most common presentations, prompting us to the initial impression of bacterial sepsis and/or necrotizing enterocolitis. Eleven associated factors for candidemia were relating to the measures and therapy in the neonatal intensive care unit, such as prolonged use of broad-spectrum antibiotics, parenteral hyperalimentation etc. All of these 18 patients were treated with intravenous amphotericin B. Six patients were given adequate total dose (> 25 mg/Kg), while 12 patients underwent inadequate treatment (3.7 +/- 2.9 mg/Kg). The 10 fatal patients belonged to the inadequate treatment group. As there is continual progress in neonatal intensive care units, candidemia is becoming an increasing common problem and which deserves attention.

Amphotericin B↗