Biomedical subjects

C S Chi

Publications and source records attributed to C S Chi.

At least 163 records · Page 9Linked to original sources

PubMedDate not supplied

Neonatal Staphylococcus aureus pancarditis: report of one case.

Due to poor feeding, decreased activity and severe abdominal distension, a 20-day old female infant was admitted to our hospital. The patient was diagnosed as having necrotizing enterocolitis and received a multiple segmental resection. During the hospitalization, recurrent sepsis and candidiasis occurred. On the 56th day of admission, tachycardia, thrombocytopenia and cardiomegaly were noted. Sudden onset of cardiac arrest occurred and a post-mortem examination revealed staphylococcal pancarditis. Pancarditis is a rare and fatal disease in newborns. When a patient with a catheter insertion shows unknown cause of thrombocytopenia and acute onset of cardiomegaly, an echocardiogram is highly suggested. Early diagnosis and proper parenteral antibiotics may be life-saving.

Acute Disease↗

PubMedDate not supplied

Still's disease: experience in 12 children.

Over a 9-year period, twelve patients (8 boys, 4 girls), from 3 to 14 years old, were diagnosed as having Still's disease. Intermittent spiking high fever, poly- or pauci- articular arthritis, and typical evanescent skin rash were the most prominent clinical features. Hemogram examinations showed that 36% of the patients had anemia, ninety-two percent had neutrophilic leukocytosis and 78% had thrombocytosis. Serologically, none had positive results of rheumatoid factor and anti-nuclear antibody. Serum ferritin level was obtained from six patients and all revealed marked elevation during active disease. C-reactive protein and erythrocyte sedimentation rate were both invariably elevated. Immunologically, elevated serum concentrations of IgG, IgA, and complements (C3, C4) were found in 33%, 20%, and 17%, respectively. Furthermore, eighty percent of patients showed an increased serum level of circulating immune complexes. Aspirin (ASA) was used in all patients, but 92% of them required non-steroid antiinflammatory drugs (NSAIDs) in combination to get a better response. Sixty-seven percent of patients needed corticosteroids to control the acute systemic manifestations. Other disease-modifying agents were also used in 33% of our patients. ASA-induced liver function impairment was found in two cases. In addition, one patient experienced an episode of upper gastrointestinal bleeding. Generally speaking, the overall prognosis was good. One patient (8%) died of internal bleeding after a needle liver biopsy.

Adolescent↗

PubMedDate not supplied

Hemorrhagic shock and encephalopathy-like syndrome: report of a case with emphasis on the pathological findings.

The case of a 5-month-old boy with sudden onset of shock, encephalopathy, watery diarrhea, bleeding tendency, and impaired hepatic and renal functions was reported. The different pathological findings as compared with the previous reports made the term "hemorrhagic shock encephalopathy-like syndrome" (HSE-like syndrome) more suitable for this case. Marked fatty metamorphosis with hemorrhage was found with a light microscope. Electron microscopy revealed flocculent densities in the mitochondria which might be a poor prognostic indicator in this syndrome. Diffuse electron-dense deposits in the peroxisomes were also seen and the significance of this finding was discussed. The patient died on the third day after admission due to multiorgan failures in spite of our aggressive management.

Brain Diseases↗

PubMedDate not supplied

Neonatal Candida albicans meningitis: report of one case.

Candida meningitis is a growing problem today. We report a 21-day-old male baby who was a victim of Candida albicans meningitis with the initial presentations of fever, mild stiff neck, poor feeding and activity. He had been treated with intravenous antibiotics and ventilator therapy prior to admission Initially, he was treated as a case of bacterial sepsis after admission with intravenous antibiotics. Due to positive cultures of cerebrospinal fluid for Candida albicans twice, intravenous amphotericin B was started from the 13th hospital day and was continued for 38 days. The successive three sets of CSF fungus culture yielded negative results and the patient was doing well without fever. Meanwhile, the brain sonogram revealed normal findings and he was discharged in a stable condition. We report this case and review some literature in an attempt to know more about this unusual disease, which is becoming more frequent as progress in intensive care grows.

Candidiasis↗

PubMedDate not supplied

Congenital syphilis: report of twelve cases.

Between 1983 and 1992 at the Taichung Veterans General Hospital, twelve congenital syphilis patients were diagnosed, according to the Centers for Disease Control (CDC) criteria. For the five boys and seven girls, ages at diagnosis ranged from 1 day to 8 months, with an average of 23 days. Among these 12 patients, four (33%) were premature. Seven patients (58%) were diagnosed in the past three years. These 12 patients were divided into inborn and outborn patient groups. Serological tests for syphilis were done in all inborn patients, but none in the outborns. All five outborn patients were symptomatic. In comparison, only one of seven inborn infants showed clinical evidence of congenital syphilis. The mean age at diagnosis was obviously older in the outborn patient group (53 days vs. 1 day). Six of ten patients (60%) had cerebrospinal fluid (CSF) abnormalities, while four of nine (44%) showed radiological abnormalities of bones. The hematologic and biochemical abnormalities compatible with congenital syphilis were also described. Penicillin was the drug of choice in ten patients. Despite adequate treatment, one patient died of respiratory failure. There has been no evidence of any signs of late congenital syphilis in those patients who survive. Congenital syphilis, a serious but preventable disease, is best treated adequately and early for minimal sequelae.

Female↗

PubMedDate not supplied

Childhood non-Hodgkin's lymphoma--results of treatment with ALL high-risk protocol.

Between August 1984 and September 1990, 13 children with non-Hodgkin's lymphoma (NHL) were treated with ALL high-risk protocol (5 with TCLSG 842 regimen and 8 with TPOG 882B protocol) at Taichung Veterans General Hospital. Diagnosis of NHL was confirmed by histology. Nine had lymphoblastic lymphoma, three had histiocytic lymphoma and one had small non-cleaved cell lymphoma, according to the Rappaport classification. After thorough clinical evaluation eight children were NHL stage IV; four were stage III; and one was stage II. All these children had received chemotherapy as acute lymphocytic leukemia (ALL) high-risk protocol for a total of three years, including central nervous system (CNS) prophylaxis. Of the 13 patients, 9 (69.2%) gained complete remission. Over-all survival rate was 46.2%, with a median interval of 36 months. The complete remission rate and survival rate for the nine children with lymphoblastic lymphoma was better at 77.7% & 66.6% respectively. Within the nonresponsive group, two failed to remit because of early withdrawal from the protocol; the other two patients were refractory to treatment. Relapse was noted in one patient. As to the side effects, neutropenic fever was the most common problem encountered (9 occurrences in 13 patients). Other major complications included severe mucositis, massive GI bleeding, intracranial thrombosis induced by l-asparaginase and tumor lysis syndrome. Childhood NHL is often of diffuse types in pathology, and most affected children have advanced diseases at diagnosis. Choosing an optimal treatment protocol is important for good treatment results.

Adolescent↗

PubMedDate not supplied

Arthrogryposis multiplex congenita: report of a case of amyoplasia.

Arthrogryposis multiplex congenita is a congenital syndrome characterized by multiple congenital joint contractures; and refers to a large heterogeneous group of disorders. We present a 40 days old male baby who has had multiple fixed contractures of joints since birth. Midline capillary hemangioma, internal rotation of bilateral shoulders, extension contracture of elbows, flexed wrists with pronation deformity, flexion and internal rotation of hips, equinovarus of feet were noted. A series of examinations for differential diagnosis including brain sonogram, echocardiogram, muscle echogram, muscle biopsy, chromosome study were performed and all showed normal findings. Amyoplasia was impressed. Early rehabilitation was arranged. Normal intelligence and a normal life span are expected.

Arthrogryposis↗

PubMedDate not supplied

Leigh syndrome presenting with dystonia: report of one case.

A 22-month-old female presented with developmental delay and dystonia. The T2 weighted image of the brain MRI showed bilateral symmetrical high signal lesions over the putamen. An increased serum lactate pyruvate ratio (29.25) and a positive oral glucose lactate stimulation test were noted. Electron microscopic examination showed abnormal mitochondrial aggregation with band cristae in the subsarcolemmal area. These findings were indicative of clinical Leigh's syndrome. However, unusually the mt DNA analysis showed a point mutation at the nucleotide position 8344.

DNA, Mitochondrial↗

PubMedDate not supplied

Neuroimage in infants and children with mitochondrial disorders.

Neuroimage studies of thirty-eight infants and children with mitochondrial disorders were reviewed: 24 ultrasound (US), 21 computed tomography (CT), and 27 magnetic resonance image (MRI) examinations were analyzed. Patients included seventeen with Leigh syndrome, two with Kearns-Sayre syndrome (KSS), one with myoclonus, epilepsy, and ragged red fibers (MERRF), one with Alpers disease, five with Menkes disease, two with fatty acid metabolic defect, two with Rett syndrome, and eight with unspecified mitochondrial disorders. KSS and MERRF tended to occur in older children, whereas Leigh syndrome, Menkes disease, and Alpers disease occurred in infants and young children. The deep cerebral nuclei and the cerebral white matter were commonly involved in Leigh syndrome and KSS. Subdural hematomas or effusions with profound cerebral atrophy was found in Alpers disease and Menkes disease. Tortuosities of basilar, Willis circle, and cerebral vessels were also noted in Menkes disease. MRI and CT examinations of Rett syndrome, fatty acid metabolic defect, and most of the unspecified mitochondrial disorders were normal. Our results indicate that neuroimage studies have characteristic findings for specific mitochondrial syndromes.

Adolescent↗

PubMedDate not supplied

Magnetic resonance imaging of herpes simplex encephalitis.

Eight brain magnetic resonance imagings (MRIs) and one spinal MRI of 7 small infants and children with herpes simplex encephalitis (HSE) were retrospectively studied. Hypointense and hyperintense areas of gray and white matters on T1- and T2- weighted images, respectively, were commonly present, with temporal lobes being the most common lesion sites. Hemorrhagic lesions were found in 4 patients (57%). Early involvement of the white matter, as early as day 4, was a common MRI finding in these patients. One patient had relapsed encephalomyelitis, whose spinal MRI showed diffuse hyperintense T2 signals from the lumbar spinal cord to the conus medullaris. All patients but one survived with major neurological sequelae. Our results indicate that MRI is a sensitive diagnostic modality in cases of HSE, and early involvement of white matter is not an uncommon MRI finding of HSE. Spinal MRI may be helpful in the diagnosis of relapsed herpes encephalomyelitis.

Brain↗

PubMedDate not supplied

Cerebellar dysgenesis in infants and children: an experience of 22 cases.

There were a total of 22 cases of cerebellar dysgenesis documented by brain sonogram, and/or brain computer-tomography scan, and/or brain magnetic resonance imaging (MRI) in our department over the past 10 years. There were ten males and twelve females. The mean age at diagnosis was 5.79 months. The follow-up period ranged from 2 days to 132 months. Seven cases were suspected upon prenatal examination. Three cases presented with isolated cerebellar hypoplasia, one with Dandy- Walker malformation and three with Joubert syndrome. Seven cases presented with cerebellar dysgenesis complicated with supratentorial brain dysgenesis. Among them, three had vermis hypoplasia with hypoplasia of the corpus callosum, 1 had vermis hypoplasia with holoprosencephaly, 1 had cerebellar hypoplasia with lissencephaly and hypoplasia of corpus callosum, 1 had vermis hypoplasia, agenesis of the corpus callosum and pachygyria, and 1 had cerebellar hypoplasia, hypoplasia of corpus callosum and midline cystic malformation. They all showed severe psychomotor retardation. Six cases showed chromosome anomalies. The neurological outcome for cases with isolated cerebellar hypoplasia was better than the outcome of the complicated cases. MRI is recommended for patients with microcephaly to check for the possibility of combined supratentorial brain dysgenesis. When performing MRI, a median sagittal view should be included. A classification for clinical approach was presented at the same time. In this retrospective study, this classification seemed to have benefits in prediction of clinical outcomes.

Cerebellum↗

PubMedDate not supplied

Lupus-like autoimmune acute hepatitis: report of one case.

In this article, a girl with an especially severe form of autoimmune acute hepatitis was successfully treated with immunosuppressive medication. This 11 year-old girl was hospitalized with the chief complaint of persistent jaundice for three months. The pathohistology of liver biopsy was chiefly composed of massive necrosis and heavy lymphoid infiltration. With the clinical findings of pericardial effusion, arthritis, positive ANA, and positive anti-dsDNA, she initially was mistaken for a case of unusual SLE complicated with hepatic involvement. Differentiating the autoimmune hepatitis from the hepatic involvement of SLE for this case is illustrated by a review of the pertinent literature and our experiences with SLE. The characteristic features of autoimmune hepatitis, the relatively low titer of anti-dsDNA, and the inherent low level of C4 finally led to the conclusion that the cause for her liver disease was in favor of autoimmune hepatitis.

Acute Disease↗

PubMedDate not supplied

Clinical analysis of 22 infants with afebrile cluster seizures.

The inclusion criteria for afebrile cluster seizures in infancy are defined as follows: (1) frequency of afebrile seizures at least 2 episodes within 72 hours; (2) seizure onset between 2 months and 3 years of age; (3) excluding febrile convulsion, central nervous system infections, status epilepticus, well-known epileptic syndromes in infancy (e.g. early myoclonic encephalopathy, early infantile epileptic encephalopathy, benign myoclonic epilepsy, infantile spasms. Lennox-Gastaut syndrome), electrolyte imbalance, watery diarrhea, head injury and intoxication. From 1986 to 1996, retrospectively and prospectively 22 patients were collected who fulfilled the above criteria. Based on whether or not a strong family history was present and a history of mild diarrhea was associated with seizure onset, they were divided into three groups: Group I, benign infantile familial convulsions (4 patients); Group II, cluster seizures with mild diarrhea in infancy (5 patients); Group III, cluster seizures without diarrhea in infancy (13 patients). Before seizure onset and during follow-up, all of the patients had normal development. The seizure pattern in all was generalized, most tonic type with duration of seizure less than five minutes in the majority. Recurrence rate was 100% in Group I and no recurrence in Group II. In 16 patients who were seizure-free over 12 months, the duration of persistence varied from 1 day to 8 months, and was shortest in Group II (range, 1 to 3 days). It was concluded that the vast majority of afebrile cluster seizures in infancy are benign in nature. Whether anticonvulsant therapy is justified must be individualized.

Child, Preschool↗

PubMedDate not supplied

Cutis marmorata telangiectatica congenita: report of one case.

Cutis marmorata telangiectatica congenita is an uncommon, congenital cutaneous lesion. It is usually present at birth, and is characterized by persistent fixed, flat, blue-violet cutaneous marmorata, telangiectasia, and phlebectasia. Frequently, there is associated skin atrophy and ulceration. There is an association with other abnormalities in at least 50% of the patients with cutis marmorata telangiectatica congenita. Thus, obtaining a thorough history and detailed physical examination are needed. We present a 38-day-old female infant with cutis marmorata telangiectatica congenita, with uneven growth of bilateral lower limbs, and review the literature on this rare lesion.

Female↗

PubMedDate not supplied

Familial primary hypomagnesemia complicated with brain atrophy and cardiomyopathy.

A 2 5/12-year-old Chinese boy was investigated for refractory seizures and psychomotor regression. His birth history was unremarkable. Generalized seizures occurred at 2 weeks of age with hypocalcemia. They recurred at 7 months of age and have become aggravated since. During hospitalization, in addition to hypocalcemia and hypomagnesemia, he was found to have hypoparathyroidism, cardiomyopathy, and brain atrophy. Excessive renal loss of magnesium, general intestinal malabsorption, or inadequate dietary intake of magnesium were excluded. He was successfully treated with oral supplements of 19-25 mmole/day of magnesium. Over a few months, he made a dramatic progress in development. His hypoparathyroidism and cardiomyopathy gradually resolved. However, intermittent seizures and psychomotor retardation persisted up to his present age of 6 3/12 years. At 4 months of age his younger sister also developed seizures and was found to have isolated hypomagnesemia. This was corrected by oral magnesium and followed by resolution of the seizure. She has developed normally up to her present age of 1 10/12 years. Both patients are currently maintained on oral magnesium oxide.

Atrophy↗

PubMedDate not supplied

Central core disease associated with scoliosis: report of one case.

A 13 year-old girl with scoliosis and central core disease is reported. She was noted to have mild psychomotor developmental delay since early infancy. Scoliosis with minimal muscle weakness was noted at about five years old. The neurological examination disclosed absent knee jerk. The spine MRI showed no significant finding. The serum CK revealed 518 U/L. The muscle biopsy obtained from the quadriceps femoris muscle showed Type 1 fiber atrophy and predominance, as is commonly seen in congenital myopathies. Under nicotinamide adenine dinucleotide dehydrogenase (NADH) and succinate dehydrogenase (SDH) stains, core structures were identified and the diagnosis of central core disease (CCD) was made. Since kyphoscoliosis usually becomes prominent as muscle weakness progresses to loss of ambulation in other myopathies, the disproportionate spinal involvement in central core disease appears to be a striking feature. We suggest that all patients with idiopathic scoliosis deserve a thorough neurological evaluation if congenital myopathies are suspected. Muscle biopsy should also be recommended for a confirmatory diagnosis even if only minimal muscle weakness present. Besides, early detection of CCD helps us to identify the population who are at a higher risk for malignant hyperthermia.

Adolescent↗

PubMedDate not supplied

Intermittent form of maple syrup urine disease: report of one case.

We present a Chinese female infant with an intermittent form of MSUD whose early development was relatively well. A total of three episodes of stupor and metabolic acidosis developed with a concurrent illness after the age of 13 months. The initial analyses of plasma amino acid and urinary organic acid were normal. Initially, an abnormal oral glucose lactate stimulation test and high signal in the bilateral globus pallidus over T2-weighted brain MRI led us to suspect a mitochondrial disorder. The final diagnosis was made after the patient died at 31 months of age.

Female↗

PubMedDate not supplied

Intoxication after ingestion of toad soup: report of two cases.

Intoxication after ingestion of toad is rare, but it results in severe morbidity and high mortality. Recently, we encountered 2 children, one a 15-month-old boy and one a 20-month-old girl, who were fed cooked toad (Bufo melanostictus) soup. The boy expired of ventricular fibrillation. The girl presented with varying degrees of A-V block with congestive heart failure which, however, was completely resolved after intensive treatment. In our experience, early identification and intervention are necessary to decrease absorption of toxins; to correct hyperkalemia. Dysrhythmia should be treated with atropine, antiarrhythmic agents, and a temporary pacemaker, in order to avoid lethal results.

Animals↗