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Biomedical subjects

G Johnson

Publications and source records attributed to G Johnson.

At least 361 records · Page 20Linked to original sources

The cardinal manifestations of Bardet-Biedl syndrome, a form of Laurence-Moon-Biedl syndrome.

To determine the interfamilial and intrafamilial variation in the expression of the Bardet-Biedl syndrome (a form of Laurence-Moon-Biedl syndrome), we looked for the five recognized features of the disorder (retinal dystrophy, obesity, polydactyly, mental retardation, and hypogonadism), plus possible renal manifestations, in some or all of 32 patients with this disorder. All 28 patients examined had severe retinal dystrophy, but only 2 had typical retinitis pigmentosa. Polydactyly was present in 18 of 31 patients, but syndactyly, brachydactyly, or both were present in all. Obesity was present in all but 1 of 25 patients. Only 13 of 32 patients were considered mentally retarded. Scores on verbal subtests of intelligence were usually lower than scores on performance tasks. Seven of eight men had small testes and genitalia, which was not due to hypogonadotropism. All 12 women studied had menstrual irregularities, and 3 had low serum estrogen levels (1 of these had hypogonadotropism, and 2 had primary gonadal failure). The remaining women who were of reproductive age had endocrinologic evidence of reproductive dysfunction. Diabetes mellitus was present in 9 of 20 patients. Renal structural or functional abnormalities were universal (n = 21), and three patients had end-stage renal failure. We conclude that the characteristic features of Bardet-Biedl syndrome are severe retinal dystrophy, dysmorphic extremities, obesity, renal abnormalities, and (in male patients only) hypogenitalism. Mental retardation, polydactyly, and hypogonadism in female patients are not necessarily present.

Female↗

Taking mental health services to the people: the effects of referral to traditional psychiatric facilities.

This study investigated the effects of establishing a locally integrated mental health team in a small New Zealand township (Mosgiel), by comparing it with a similar township (Port Chalmers) without such established services. The rate of referral for out-of-district public and private inpatient and outpatient care was taken as an inverse measure of the strength of community resources to cope with psychiatric problems at the local level. In terms of referrals for inpatient care it was found that increased locally available mental health services did not reduce the number of onward referrals. However, length of hospital stay was significantly less. The total number of referrals to Dunedin for outpatient care also showed no significant change over time. The effect in this instance seems to be qualitative in that the proportion of first time referrals decreased while referrals tended to increase. It was also found that the lack of community mental health teams resulted in more self referrals to private health care. The appropriateness of this development needs further investigation. The presence of such a team is associated with a refinement in general practitioner referring practices.

Community Mental Health Services↗

Evaluation of electrode polarity on defibrillation efficacy.

The effect of electrode polarity on defibrillation thresholds in humans is unknown. This prospective, randomized evaluation of electrode polarity on defibrillation thresholds was performed in 21 survivors of ventricular fibrillation (VF) undergoing cardiac surgery. Defibrillation was always performed with 2 identical large rectangular, wire mesh electrodes positioned over the anterior wall of the right ventricle and the posterolateral wall of the left ventricle. The initial electrode polarity for the left ventricular (LV) electrode was chosen randomly for determination of the defibrillation threshold. Subsequently, electrode polarity was reversed. The defibrillation threshold was defined as the lowest pulse amplitude that would effectively terminate VF with a single discharge delivered 10 seconds after initiation of an episode of VF with alternating current. For each defibrillation pulse, voltage, current, resistance and delivered energy were recorded. Of the 21 patients, 15 (71%) had a lower defibrillation threshold when the LV electrode was positive, 2 patients (10%) had a lower defibrillation threshold when the LV electrode was negative and 4 patients (19%) had equal defibrillation thresholds (within 0.5 J) regardless of polarity. The mean leading edge defibrillation threshold voltage was 370 +/- 88 volts when the LV electrode was negative and 320 +/- 109 volts (14% less) when the LV electrode was positive (p = 0.014). Mean leading edge defibrillation threshold current was 9.3 +/- 3.1 amps when the LV electrode was negative compared to 7.7 +/- 3.1 amps (17% less) when the LV electrode was positive (p = 0.0033). There were no differences in resistance with the 2 configurations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Precise relaxation time measurements of normal-appearing white matter in inflammatory central nervous system disease.

Precise relaxation time (RT) measurements have been made, with a standard deviation of 3% for T1 and T2 in white matter in normal volunteers. This sets an upper limit to the instrumental random errors (imprecision). Achieving this precision requires careful adjustment and use of the imager. The wide variation in RTs seen by other workers may be in part due to larger instrumental errors. We have measured RTs (both T1 and T2) in normal-appearing white matter in 16 normal controls and patients with multiple sclerosis (MS, 18), systemic lupus erythematosis (SLE, 16) and cerebral sarcoidosis (8). Both RTs were significantly higher in MS than in other patient groups and controls (P less than .05), possibly caused by microscopic lesions. T2 was elevated in SLE patients relative to controls and sarcoidosis patients (P less than .05), possibly because of microhemorrhages. Lesion RTs were abnormal but more variable and no significant differences between diseases were found.

Adolescent↗

The influence of perfusate viscosity, RBC deformability and drag on the function of an isolated perfused rat kidney.

There is increasing clinical interest in improving blood rheology to optimize organ function, but studies correlating the two are scarce. To study this, rat kidneys were perfused in vitro at 37 degrees C at a constant mean renal arterial pressure of 160 mm Hg. The perfusate consisted of an oxygenated Krebs HCO3 buffer containing 1 mg/ml glucose, 0.5 mg/ml creatinine, amino acids, [3H]inulin (marker for GFR), 2.5 g/dl albumin, and 10 or 20% hematocrit. In some experiments, RBC were made nondeformable by heating at 50 degrees C for 20 min. Deformability was measured by an ektacytometer. In other experiments, 0.001% of an anionic polyacrylamide (Separan), a drag-reducing agent, was added to the perfusate. Viscosity was measured with a cone and plate viscometer, [Na+] with a flame spectrophotometer, and perfusate flow with a Brooks in-line flowmeter. Other functional parameters, GFR, urine flow, RPF, and reabsorption of sodium and water, were also measured. The results indicate that (1) making RBC nondeformable or increasing viscosity by increasing hematocrit reduces renal function, and (2) addition of a drag-reducing agent improves renal function at 20% hematocrit. We conclude that rheologic manipulation of a perfusate solution can alter flow and renal function.

Absorption↗

The effect of minoxidil on endothelial cells in vitro.

Minoxidil - a therapeutic agent used in the control of hypertension and which has been implicated in modulation of hair follicle activity - has been shown to influence certain aspects of the physiology of cultured endothelial cells. It suspends cell proliferation in a reversible manner, appears to influence cell morphology in a dose dependent way, does not significantly alter (qualitatively) protein synthesis and does not inhibit cell migration. These results demonstrate similarities between endothelial cells and keratinocytes and dermal fibroblasts and differences between endothelial cells and dermal papilla cells, follicular root sheath fibroblasts and neutrophils in their response to this agent.

Animals↗

Prospective comparison of sequential pulse and single pulse defibrillation with use of two different clinically available systems.

Sixteen out-of-hospital survivors of ventricular fibrillation underwent a prospective, randomized, intraoperative comparison of sequential pulse and single pulse defibrillation with use of two distinct electrode systems and waveform shapes currently available for clinical use. Defibrillation was tested alternately with either the single pulse or the sequential pulse system 10 s into an episode of ventricular fibrillation. Sequential pulse defibrillation was performed with two 4 ms truncated exponential pulses of constant duration delivered to three equally spaced oval epicardial patch electrodes composed of concentric coils. The posterior left ventricular electrode served as the common cathode. The first anode was over the anterior right ventricle and the second anode was over the anterior left ventricle. Single pulse defibrillation was performed with the standard intracardiac defibrillation system with use of a single truncated exponential pulse with a fixed 65% tilt delivered across two rectangular, wire mesh epicardial patch electrodes positioned over the anterior right ventricle and posterolateral left ventricle. During defibrillation threshold determination, voltage and current waveforms were recorded and used to determine pulsing resistance and delivered and stored energy. Average defibrillation threshold leading edge voltage for the single pulse technique was 273 +/- 101 V compared with 246 +/- 67 V (11% less) for the sequential pulse technique (p = 0.136). Defibrillation threshold leading edge current for the single pulse technique was 6.7 +/- 2.5 A compared with 5.2 +/- 1.7 A (29% less) for the sequential pulse method (p = 0.005). The defibrillation threshold delivered energy was 5.6 +/- 4.0 J for the single pulse technique and 3.5 +/- 1.8 J (38% less) for the sequential pulse technique (p = 0.021).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

A prospective randomized evaluation of biphasic versus monophasic waveform pulses on defibrillation efficacy in humans.

Biphasic waveforms have been suggested as a superior waveform for ventricular defibrillation. To test this premise, a prospective randomized intraoperative evaluation of defibrillation efficacy of monophasic and biphasic waveform pulses was performed in 22 survivors of out of hospital ventricular fibrillation who were undergoing cardiac surgery for implantation of an automatic defibrillator. The initial waveform used in a patient for defibrillation testing, either monophasic or biphasic, was randomly selected. Subsequently, each patient served as his or her own control for defibrillation testing of the other waveform. The defibrillation threshold was defined as the lowest pulse amplitude that would effectively terminate ventricular fibrillation with a single discharge delivered 10 s after initiation of an episode of ventricular fibrillation induced with alternating current. Each defibrillation pulse was recorded oscilloscopically, and defibrillation pulse voltage, current, resistance and stored energy were measured. Fifteen (68%) of the 22 patients had a lower defibrillation threshold with the biphasic pulse, 3 (14%) had a lower threshold with the monophasic pulse and 4 (18%) had equal defibrillation thresholds (within 1.0 J) regardless of waveform. The mean leading edge defibrillation threshold voltage was 317 +/- 105 V when the monophasic pulse was used and 267 +/- 102 V (16% less) when the biphasic pulse was used (p = 0.008). Mean leading edge defibrillation threshold current was 7.9 +/- 3.7 A when the monophasic pulse was used and 6.8 +/- 3.8 A (14% less) when the biphasic pulse was used (p = 0.051).(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

A prospective, randomized evaluation of effect of ventricular fibrillation duration on defibrillation thresholds in humans.

The effect of ventricular fibrillation duration in humans on defibrillation efficacy as it pertains to the time of intervention of an automatic implantable defibrillator is unknown. If a difference in defibrillation efficacy exists in the early period after ventricular fibrillation onset, it may affect algorithms used by antiarrhythmic devices for arrhythmia detection and therapy. Therefore, a prospective, randomized evaluation was performed of the effect of ventricular fibrillation durations of 10 s and 20 s on defibrillation thresholds in 10 survivors of sudden cardiac arrest undergoing implantation of an automatic cardioverter defibrillator. The initial duration of ventricular fibrillation was chosen randomly. Subsequently, each patient served as his or her own control for the alternate duration of ventricular fibrillation to that chosen initially. The mean leading edge defibrillation threshold voltage was 411 +/- 114 V when ventricular fibrillation persisted for 10 s and 419 +/- 125 V when it persisted for 20 s (p = 0.73). The mean defibrillation threshold current was 11.4 +/- 2.8 A when ventricular fibrillation persisted for 10 s and 11.4 +/- 3.2 A when it persisted for 20 s (p = 0.97). The delivered energy defibrillation threshold was 11.5 +/- 5.9 J when ventricular fibrillation persisted for 10 s and 12.0 +/- 6.9 J when it persisted for 20 s (p = 0.67). These findings show that the defibrillation threshold does not change between 10 and 20 s of ventricular fibrillation in out-of-hospital survivors of cardiac arrest at the time of surgical implantation of an automatic defibrillator. The data may have influence on the programming of defibrillator detection algorithms.

Algorithms↗

A mechanical study of six digital pulley reconstruction techniques: Part I. Mechanical effectiveness.

A laboratory study on fresh-frozen human cadaver limbs compared six types of flexor tendon pulley reconstruction in the fingers. The reconstructions used were those described by (1) Bunnell, (2) Karev, (3) Weilby, and (4) Lister, and two types developed by us that have not been previously described. The pulleys were studied in specific configurations to determine their effectiveness in transforming tendon excursion into finger flexion. In each finger, comparison was made between the amount of tendon excursion required for full flexion with an intact pulley system versus the various types of reconstructed pulleys. The ratio of these two values was defined as the mechanical effectiveness of the pulley reconstruction. The Karve reconstruction was most effective at 108.2%, which was significantly different from the other five reconstructions (p less than 0.01). The remaining reconstructions ranged in effectiveness from 80.0% to 88.5%.

Aged↗

A mechanical study of six digital pulley reconstruction techniques: Part II. Strength of individual reconstructions.

A laboratory study was done on fresh-frozen human cadaver limbs, using six types of pulley reconstructions about the flexor tendons of the fingers. The reconstructions used were those described by (1) Bunnell, (2) Karev, (3) Weilby, and (4) Lister, and two types developed by us that have not been previously described. The pulleys were tested at constant strain rate to failure with the peak force recorded as the breaking strength. A total of 385 reconstructed pulleys were tested and the results were analyzed statistically. A new "loop and one half" pulley reconstruction was significantly stronger than the other five reconstructions (p less than 0.01) with an average load to failure of 22.5 kilograms-force. The other pulley reconstructions varied in average breaking strength from 2.8 kilograms-force to 18.4 kilograms-force.

Biomechanical Phenomena↗

Exploration of phenyl-spaced 2-amino-(5-9)-phosphonoalkanoic acids as competitive N-methyl-D-aspartic acid antagonists.

To investigate the preferred spatial relationship of the distal phosphonic acid to the alpha-amino acid group of the established competitive N-methyl-D-aspartic acid (NMDA) antagonists APH (1) and APV (2), we have prepared a series of ortho-, meta-, and para-substituted (phosphonoalkyl)phenylglycine and -phenylalanine derivatives. With use of a [3H]CPP receptor binding assay, significant binding activity was observed to be critically dependent on both the position of substitution and length of alkyl spacing groups. Two compounds, 4-(phosphonomethyl)-phenylglycine (6, PD 129635) and 3-(phosphonomethyl)phenylalanine (15, PD 130527), displayed receptor-binding affinity comparable to that of APH. Like APH, these compounds were also effective in antagonizing both the proconvulsant and lethal action of NMDA-administered retrobulbar in the mouse. Data are also provided which compare directly the binding efficacy of these compounds against that disclosed recently for the related NMDA antagonist 18 (NPC 451). A preliminary comparison of the structures showing good receptor-binding affinity and in vivo antagonist activity suggests that the NMDA receptor prefers a "folded" rather than "extended" conformation.

Amino Acids↗

Evaluation and synthesis of aminohydroxyisoxazoles and pyrazoles as potential glycine agonists.

Except for structurally similar small amino acids, such as alanine, beta-alanine, and serine, compounds acting as glycine-receptor agonists are an unknown class of pharmacological agents. To investigate the potential of small, substituted heterocycles to act as glycine agonists, we have evaluated the similarities between glycine and a series of hydroxy- and amino-substituted pyrazoles and isoxazoles through complementary molecular modeling techniques. Using a "scorecard approach" to determine the overall similarity of projected agonist structures to glycine, we prioritized synthesis and subsequently prepared several novel derivatives. The biological activity of these compounds was compared to that of glycine by using a [3H]strychnine-mediated glycine receptor binding assay. Despite the close similarity in the calculated parameters when compared to glycine, no significant receptor-binding activity was observed for the targeted analogues. These results illustrate the structurally exacting nature of the glycine receptor.

Animals↗

A multi-element proportional counter for radiation protection measurements.

A detector incorporating about 300 individual counting volumes is described, and the results of performance tests are reported. The device can be employed for a direct measurement of the dose equivalent in an unspecified radiation field on the basis of the lineal energy spectrum in 1-micron diameter tissue regions. It is substantially smaller than a conventional tissue equivalent proportional counter yielding the same counting rate and may be useful for measurements in phantoms.

Radiometry↗

Effect of Growth Temperature on the Biosynthesis of Chloroplastic Galactosyldiacylglycerol Molecular Species in Brassica napus Leaves.

Brassica napus leaves developed at low temperature display rapid in situ desaturation of monogalactosyldiacylglycerol (MGDG) fatty acids leading to the production of hexadecatrienoic/linolenic acid. This was shown by radioactivity-tracer experiments to occur via a sequence of desaturations proceeding from the initially synthesized palmitic/oleic acid molecular species to palmitic/linoleic acid, palmitoleic/linoleic acid, hexadecadienoic/linoleic acid, hexadecadienoic/linolenic acid, and finally to hexadecatrienoic/linolenic acid. The results suggest that there is increased activity in all five desaturation steps in leaves developed at low temperatures. Labeling data also indicate that there is another pool of MGDG which is more slowly desaturated before galactosylation to digalactosyldiacylglycerol (DGDG). Our data further suggest that relative rates of galactosylation of chloroplastic and cytosolic MGDG molecular species may regulate the final amounts of chloroplastic and cytosolic MGDG and DGDG in the leaf. We have proposed a model for chloroplastic biosynthesis and desaturation of galactosyldiacylglycerols in the leaves of Brassica napus, a 16:3 plant.

Journal Article↗

Alterations of the anterior lens capsule associated with climatic keratopathy.

We describe changes in the anterior lens capsules of older people in Somalia, a country which is close to the equator and with large areas of sand, often highly reflective of sunlight. The capsule changes are confined to the central pupillary area. In order of apparently increasing severity they consist of a white opalescence ('frosting'), an elevation in front of the contour of the rest of the lens to form a plateau, and a 'bag' or herniation of the lens capsule through the pupil. Plateau and bagging taken together are strongly associated with climatic keratopathy (and by inference with reflected ultraviolet exposure), weakly associated with exfoliation syndrome, inversely related to the degree of cataract, and interfere severely with vision.

Adult↗

Quantitative magnetic resonance imaging in multiple sclerosis: the effect of high dose intravenous methylprednisolone.

Magnetic resonance imaging was performed on 50 patients with clinically definite or probable multiple sclerosis before and 15 days after starting treatment with intravenous methylprednisolone (0.5 g daily for 5 days). Scans were abnormal in 49 patients. New lesions had appeared on the second scan in nine individuals and in seven a single pre-existing lesion appeared to have become smaller but in no case were lesions seen to disappear. Two patients showed both reduction in the size of an abnormal area and development of a single new lesion indicating that corticosteroids do not appear rapidly to alter the process underlying plaque formation. Measurements of relaxation times were performed in 12 randomly selected patients. All showed elevated values in normal appearing white matter but not cortex before treatment compared with 18 healthy controls. After treatment a significant decrease of T1 and T2 was observed in cortex, and of T1 alone in normal appearing white matter. No significant change could be detected within lesions, a finding attributed to the wide range of relaxation values observed at these sites before treatment. Since brain water content is increased in normal appearing white matter of multiple sclerosis patients, and is significantly reduced by high-dose methylprednisolone, resolution of oedema may contribute to the rapid spontaneous or corticosteroid induced symptomatic recovery that characterises the disease in its early stages.

Adult↗

Infrequency of blacks among patients having carotid endarterectomy.

We reviewed demographic data on patients having 2,256 carotid endarterectomies in eight large hospitals in North Carolina to determine the frequency of blacks among these patients. Blacks comprised only 4.6% of the patients having carotid endarterectomy even though they comprised 26% of all patients discharged and 22% of the general population of the state. Data from the National Inpatient Profile of the Commission on Professional and Hospital Activities, which represents patients discharged from short-term, nonfederal hospitals throughout the United States, show that nationwide, blacks comprise only 2.7% of the patients having carotid endarterectomy, whereas they comprise 12.0% of all patients discharged, 12.1% of the general population, and 10.7% of patients discharged following Class I surgical procedures. Blacks have only 67 carotid endarterectomies per 100,000 patients discharged; this rate is five or more times higher in whites. Among black patients having carotid endarterectomy, women predominate, whereas men predominate among white patients having carotid endarterectomy (p = 0.006). The underrepresentation of blacks among patients having carotid endarterectomy lends support to the concept that carotid vascular disease in blacks is distributed intracranially rather than extracranially as opposed to the extracranial rather than intracranial distribution in whites.

Adult↗