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Biomedical subjects

C Young

Publications and source records attributed to C Young.

At least 307 records · Page 17Linked to original sources

Hereditary progressive dystonia with marked diurnal fluctuation (Segawa syndrome) in Taiwan.

Since 1988, we have diagnosed 6 cases of hereditary progressive dystonia with marked diurnal fluctuation (HPD) in Taiwan. All cases presented with clinical features similar to those described by Segawa. They consisted of four sporadic and two familial cases. The age at onset ranged from 18 months to 8 years. There is a female predominance of 4:2. All of them showed mild postural tremor and postural dystonia manifested initially by flexion-inversion of a foot. However, unlike Segawa's description, side preference to the right (4:2) was noticed. Neck and axial muscles were not or were minimally involved, except a case presenting with retrocollis and tilting of the neck. These symptoms showed remarkable diurnal fluctuation which became aggravated towards the evening and alleviated in the morning or after rest. Response to L-dopa was dramatic, independent of the duration of illness, and no adverse effect of L-dopa has been observed. Our experience suggested that 10 mg/kg/day of L-dopa may be an optimally effective dose for treatment of patients with HPD. Neurophysiological, neuroradiological and biochemical studies were all normal except in one case who showed prolonged somatosensory potential latencies and white matter changes on MRI. Change of dopamine and its metabolites in CSF, plasma and urine had been investigated in one case.

Age of Onset↗

Precocious puberty in a case with probable Angelman syndrome.

The authors report a 9-year-old girl with mid-facial hypoplasia, maxillary hypoplasia, prognathia, microbrachycephaly, mouth opening and protruding tongue. She also had psychomotor retardation such as mental retardation and speech delay. Frequent laughter fits and seizure disorder was also noted. Although the high resolution chromosome study failed to demonstrate any deletion of chromosome 15q, the clinical picture was compatible with Angelman syndrome. Breast development at the age of six and rapid progression of bone age was noted at follow up. After a series of examinations, the diagnosis of gonadotropin-dependent precocious puberty was made. MRI of brain revealed an intermediate cyst in the pituitary gland and slightly enlarged pineal gland. However, serum alpha-fetoprotein and beta-HCG were undetectable and the size of the pineal gland remained the same at the 1-year follow-up. She was treated with long-acting GnRH analogue and valproic acid. The combination of precocious puberty and Angelman syndrome has not been reported before and such association needs further experience for clarification.

Angelman Syndrome↗

Relationship of a dystrophin-associated glycoprotein to junctional acetylcholine receptor clusters in rat skeletal muscle.

The relationship of a member of the transmembrane dystrophin-associated glycoprotein (DAG) complex to acetylcholine receptors (AChRs) was investigated using immunofluorescence techniques at rat neuromuscular junctions (NMJs) viewed en face. These results were compared with those from a similar previous study of dystrophin and an autosomal homologue (utrophin or dystrophin-related protein, DRP) (Bewick et al. Neuro Report 1992; 3: 857-860). The region of highest 43 K DAG (43DAG) labelling projected beyond the AChRs by approximately 0.3 microns, as does that for dystrophin. By contrast DRP labelling precisely co-localizes with the AChRs. These results suggest that at the NMJ, the region of high 43DAG concentration encompasses the area of highest intensity labelling for both DRP and dystrophin.

Amino Acid Sequence↗

Impedance cardiography: a bridge between research and clinical practice in the treatment of heart failure.

This paper proposes that impedance cardiography could narrow the gap between heart failure clinical research and the appropriate and timely application of new knowledge and technology in the care of patients with heart failure. Research variables and measurement strategies, identified in the heart failure scientific literature between 1994-1999, are compared to measures that can be obtained through a cost effective, noninvasive technology--impedance cardiography. Emphasis is placed on case reports that illustrate how "real time" evaluation and trending of key hemodynamic variables through impedance cardiography monitoring has been used to apply state of the science knowledge and technology to improve the inpatient and outpatient management of care for patients with severe heart failure. (c)2000 by CHF, Inc.

Journal Article↗

Agenesis of internal carotid artery associated with symptomatic partial epilepsy.

An 8-year-old Chinese girl had a first episode of symptomatic partial epilepsy at age 2 years. Despite regular treatment with antiepileptic drugs (AEDs), the seizures remitted and exacerbated. Epileptiform EEG discharges were evident, arising from the left centroparietal region. Magnetic resonance imaging (MRI) and computed tomography (CT) scan of the temporal bone and cerebral angiography demonstrated absence of the left internal carotid artery and ipsilateral bony carotid canal. Perfusion brain single photon emission CT (SPECT) with [99mTc] HMPAO showed a perfusion defect at the left posterior temporoparietal lobe. Brain ischemia due to agenesis of the left internal carotid artery (ICA) was speculated as the cause of the partial epilepsy.

Carotid Artery, Internal↗

Radiosurgery with unflattened 6-MV photon beams.

One of the major drawbacks to doing stereotactic radiosurgery with a linear accelerator is the long time required to deliver the target dose. Single fractions of 25 Gy delivered at the isocenter and at depth in the skull may require beam times in excess of 15 min for a typical linear accelerator with a maximum dose rate of 250 cGy/min in tissue. In an effort to decrease the treatment time for this technique, the flattening filter has been removed from an AECL Therac-6 linear accelerator and the characteristics of the resulting beam have been measured. Flatness is acceptable for the field sizes used with this technique and the dose rate is increased by a factor of 2.75.

Brain Diseases↗

A randomized controlled trial of a computer-based physician workstation in an outpatient setting: implementation barriers to outcome evaluation.

OBJECTIVE: A research prototype Physician Workstation (PWS) incorporating a graphical user interface and a drug ordering module was compared with the existing hospital information system in an academic Veterans Administration General Medical Clinic. Physicians in the intervention group received recommendations for drug substitutions to reduce costs and were alerted to potential drug interactions. The objective was to evaluate the effect of the PWS on user satisfaction, on health-related outcomes, and on costs. DESIGN: A one-year, two-period, randomized controlled trial with 37 subjects. MEASUREMENTS: Differences in the reliance on noncomputer sources of information, in user satisfaction, in the cost of prescribed medications, and in the rate of clinically relevant drug interactions were assessed. RESULTS: The study subjects logged onto the workstation an average of 6.53 times per provider and used it to generate 2.8% of prescriptions during the intervention period. On a five-point scale (5 = very satisfied, 1 = very dissatisfied), user satisfaction declined in the PWS group (3.44 to 2.98 p = 0.008), and increased in the control group (3.23 to 3.72, p < 0.0001). CONCLUSION: The intervention physicians did not use the PWS frequently enough to influence information-seeking behavior, health outcomes, or cost. The study design did not determine whether the poor usage resulted from satisfaction with the control system, problems using the PWS intervention, or the functions provided by the PWS intervention. Evaluative studies should include provisions to improve the chance of successful implementation as well as to yield maximum information if a negative study occurs.

Adult↗

Total parenteral nutrition-induced steatosis: reversal by parenteral lipid infusion.

Prolonged use of total parenteral nutrition (TPN) may be associated with hepatic complications, primarily steatosis and cholestasis. A case is reported of an 18-year-old woman with chronic idiopathic intestinal pseudo-obstruction syndrome who was on prolonged home parenteral nutrition without lipid supplementation and developed steatosis. This finding was reversed by addition of lipid emulsion, at a dose of 0.5 g/kg/day, to the parenteral nutrition solution. The lack of lipid supplementation as a possible cause of steatosis, as well as other mechanisms of liver steatosis associated with TPN, are discussed.

Adolescent↗

Anterior cruciate ligament insufficiency: a dynamic three-dimensional motion analysis.

An electrogoniometer, capable of measuring all six degrees of freedom of knee movement, was developed in our laboratory. This Instrumented Spatial Linkage system was then used to generate data for a three-dimensional assessment of 20 ACL deficient and 30 normal subjects during level walking. The system proved to be a viable method for describing total knee motion. We found the ACL deficient knees to have increased anterior/posterior translation and significant pattern differences compared to the controls. The results of this study may have implications in the treatment of the ACL deficient patient.

Adult↗

Managed competition in practice: 'value purchasing' by fourteen employers.

Many large U.S. companies have transformed their procurement of health benefits in the 1990s by combining the principles of managed competition with other business tactics to create a business-savvy hybrid of the private sector's own design, often referred to as "value purchasing." Until recently, few policymakers or health care observers believed that large firms would be a force in health system reform. Yet to implement value purchasing, the large companies in this study created new organizational forms, provided employees with financial incentives to select low-cost health plans, and used business tactics such as competitive bidding to negotiate more favorable rates and to improve quality among health plans. The financial results were impressive for the companies studied. In addition, the companies' demands on the health care delivery system are multiplying as the interface between business firms and health care organizations changes. These demands will only increase as the practices we found become more widespread.

Community Participation↗

Nurses' attire in a special hospital: perceptions of patients and staff.

Nurses' uniforms have been the subject of ongoing debate in many areas. Following the Ashworth Hospital inquiry, two wards at the hospital piloted the wearing of casual clothes by nurses. Patients and nurses in these wards completed questionnaires to evaluate the effects of the change. The results showed that the patients felt casual clothes helped remove a 'them and us' distinction and enhanced nurse/patient relationships. Nurses found casual clothes to be more comfortable and practical. Although concern was expressed that, without uniform, nurses might not be readily identifiable in an emergency situation, the change to casual clothing was generally viewed positively by both patients and nurses.

Clothing↗

A comparative study of anorganic xenogenic bone and autogenous bone implants for bone regeneration in rabbits.

The aim of this study was to investigate the possible use and ultimate fate of anorganic xenogenic bone for the restoration of defects in the maxillae and mandibles of adult rabbits. Furthermore, anorganic xenogenic bone was compared with implanted autogenous bone particles with regard to the response of the surrounding connective tissue and possible resorption of these implants by multinucleated cells. Results showed that after 12 weeks, the implanted autogenous bone was actively resorbed by multinucleated cells, and new bone was formed in close apposition to the particles. In contrast, implanted anorganic xenogenic bone was degraded to a much lesser extent, and new bone was seen adjacent to the anorganic bone particles without signs of resorption. Further long-term studies are needed to determine whether anorganic xenogenic bone may be regarded as a resorbable material and whether any side effects occur as a result of this material's tendency to linger on in the recipient bed.

Animals↗

Persistent hypertransaminasemia as the presenting findings of muscular dystrophy in childhood.

Prolonged elevation of the serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) is often attributed to hepatic diseases. However, these enzymes are also present in a variety of extrahepatic tissues, including skeletal muscle. Five children (all boys) were referred to the pediatric department of the National Taiwan University Hospital because of persistent elevation of serum aminotransferase activities. The ages of these children were between 4 months and 5.5 years. The neurological findings were all not remarkable. The initial ALT and AST values were 114-581 U/L and 183-700 U/L, respectively. Serum creatine kinase was checked first after 0 to 30 months follow-up and found to be markedly elevated (range, 10,557 U/L to 62,508 U/L). Muscle biopsies in the five cases all showed degenerating and regenerating myofibers with interstitial fibrosis. In Cases 3, 4 and 5, complete absence of dystrophin immunoreactivity was found. Genetic studies showed deletions in the DMD gene (exons 45-48 in case 2 and 49-50 in case 4). This experience indicates that occult muscle diseases should be taken into account in patients with unexplained long-lasting hypertransaminasemia and therefore measurement of serum creatine kinase activity and muscle biopsy should be done early for the correct diagnosis of muscular dystrophy.

Alanine Transaminase↗

Difficult cases in heart failure: Atrioventricular interval optimization utilizing thoracic electrical bioimpedance.

The authors describe the use of thoracic electrical bioimpedance, a noninvasive, simple, low risk technique to optimize atrioventricular delay in a patient with a dual chamber pacemaker, right ventricular infarct, and symptoms NYHA functional class III-IV. Optimization of the atrioventricular interval was associated with improvement of hemodynamic parameters and resolution of the symptoms of heart failure. The application of noninvasive hemodynamic parameters might have a great deal of impact in the management of patients with several cardiovascular diseases. Multicenter studies are underway. (c)1999 by CHF, Inc.

Journal Article↗

Factors affecting outcome of diabetic patients with foot ulcers or gangrene.

This paper reports the outcome in 205 diabetic patients with foot ulcers or gangrene. The circulation to the foot was assessed using a radioisotope clearance test to measure the skin perfusion pressure (SPP) and skin vascular resistance (SVR) in the foot. Factors important in predicting initial healing were the age of the patient, SPP and SVR but not the ankle pressure index. The factors predicting death during 6-42 months follow-up were age and initial SPP; half the patients had died or under-gone amputation at 22 months. The information from the radioisotope clearance test is important in helping determine appropriate management for these patients and provides information not available from other methods, such as the ankle pressure measurement.

Actuarial Analysis↗