Impact of rehabilitation on psychological distress: gender differences.
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Biomedical subjects
Publications and source records attributed to R T Smith.
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We report familial amyloidotic polyneuropathy in a pedigree of German ancestry residing in New Jersey. Eight affected subjects presented in the third to seventh decade with carpal tunnel syndrome (CTS) and one subject presented with vitreous opacification. Transmission was autosomal dominant and survival was prolonged. Affected subjects were heterozygous for a novel mutation in serum transthyretin (TTR), resulting in an asparagine for lysine substitution at residue 70 of the TTR monomer. We report two methods for rapid identification of the mutation based on the polymerase chain reaction. This pedigree further emphasizes the evolving phenotypic and genotypic heterogeneity of the transthyretinopathies. Familial or sporadic CTS or unexplained vitreous opacification suggest the possibility of TTR amyloidosis and should prompt a search for TTR mutations.
A new modified surgical approach for the division of posterior septal accessory pathways is described. This method incorporates some of the desirable components of previously reported techniques, while eliminating difficult and unreliable aspects of those same techniques. Interestingly this procedure was initially illustrated by Sealy and Mikat in 1983, although it has not been used clinically until now. The recognized intent of this dissection is to totally separate atrial and ventricular structures within the posterior septal space so that all accessory pathways encountered are permanently interrupted.
The properties of a peptide-transport system in rabbit enterocyte basolateral membrane were examined with glycyl-L-proline as the substrate. Basolateral-membrane vesicles prepared from rabbit proximal intestine were characterized in terms of both purity and orientation. Marker-enzyme assays show that the basolateral-membrane marker, ouabain-sensitive K(+)-activated phosphatase, is enriched 17-fold with respect to the initial homogenate. The activities of enzymes used as markers for other membranes and organelles are low, and contamination of the final membrane fraction with these is minimal. The use of immunoblotting techniques further confirms the absence of brush-border-membrane contamination. Proteins in the basolateral-membrane vesicle preparation gave no cross-reaction with antibodies against the 140 kDa antigen and the Na+/glucose-symport protein, markers specific to the brush-border membrane of the enterocyte. Conversely, antibodies raised against the classical basolateral-membrane marker, the RLA class I histocompatibility complex, reacted strongly with a 43 kDa basolateral-membrane protein. The orientation of the basolateral-membrane vesicles was shown to be predominantly inside-out on determination by two independent criteria. The uptake of [1-14C]glycyl-L-proline by these vesicles is stimulated by the presence of an inwardly directed pH gradient, and this stimulation can be abolished by the proton ionophores carbonyl cyanide p-trichloromethoxyphenylhydrazone (CCCP) and tetrachlorotrifluoromethylbenzimidazole (TTFB). Transport is also inhibited by HgCl2, thimerosal, Na+ and other glycyl dipeptides.
The distribution of fluorescein between the anterior chamber and the anterior vitreous was measured in two groups of patients after oral administration: group I, extracapsular cataract extraction (ECCE) patients with intact capsule and posterior chamber intraocular lens (PC IOL) (n = 12); group II, intracapsular cataract extraction (ICCE) patients with anterior chamber IOL (AC IOL) (n = 13). The fluorescein concentrations were measured by fluorophotometry and the penetration ratios were calculated. The penetration of fluorescein into the anterior vitreous was significantly less in the ECCE group (group I, penetration ratio = 2.03 +/- 1.00 X 10(-3) min-1; group II, penetration ratio = 5.99 +/- 4.89, X 10(-3) min-1, P less than 0.01). The authors concluded that in ECCE versus ICCE a significantly smaller proportion of fluorescein is found in the anterior vitreous relative to the aqueous after passage through the blood-aqueous barrier. This suggests a barrier to posterior movement of other molecules that may initially gain access to the eye in the anterior segment (e.g., prostaglandins).
Drug efficacy and pharmacokinetics were assessed in 63 patients, aged 5 days to 30 years (mean 8 years), who received flecainide acetate for control of resistant arrhythmias. Doses of flecainide ranged from 59 to 225 mg/m2 body surface area per day (mean 141) in divided doses every 8 to 12 h and serum trough levels ranged from 0.10 to 0.99 micrograms/ml (mean 0.36). Flecainide controlled or partially controlled arrhythmia in 53 (84%) of the 63 patients: 7 of 7 patients who had the permanent form of junctional reciprocating tachycardia, 12 of 13 who had an atrial ectopic tachycardia, 10 of 10 who had ventricular tachycardia and 18 of 25 patients who had reentrant supraventricular tachycardia. Five of seven patients who had the latter arrhythmia were unsuccessfully treated with flecainide. They had Wolff-Parkinson-White syndrome and developed asymptomatic, incessant, slower orthodromic reciprocating tachycardia while receiving the drug. Transient blurred vision was reported in three patients and two patients had transient hyperactivity. No significant hemodynamic side effects were seen in any patient. Twenty-five patients underwent oral pharmacokinetic investigation. Young infants (less than 1 year of age) had a mean plasma elimination half-life (t 1/2) approximating that (11 to 12 h) found in older children and healthy adults; children aged 1 to 12 years had a shorter mean t 1/2 of 8 h. Dosing schedules based on milligrams per square meter body surface area correlated better with plasma flecainide levels than did dosing based on milligrams per kilogram body weight.(ABSTRACT TRUNCATED AT 250 WORDS)
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Forty-one children (26 weeks gestational age to 20 years) with drug-resistant supraventricular tachycardia were treated with oral encainide, and 29 were followed for 3 to 34 months (mean 15). Diagnoses obtained by electrocardiographic criteria (23 patients) or electrophysiologic testing (18 patients) included permanent junctional reciprocating tachycardia in 15 children, paroxysmal atrioventricular reciprocating tachycardias (AVRT) in 13, atrial ectopic tachycardia in 4, atrial flutter in 1, chaotic atrial tachycardia in 5 and junctional ectopic tachycardia in 3. Encainide was completely effective in 54% (22 of 41 study patients) and partially effective in an additional 24% (10 of 41 patients), when combined with propranolol or verapamil. Within 1 month, 13 (32%) discontinued encainide for inefficacy or intolerance. Encainide was most effective in the treatment of permanent junctional reciprocating tachycardia (60% effective) and AVRT (69% effective). It controlled only 40% of primary atrial tachycardias. Encainide was well tolerated on a long-term basis in patients not experiencing symptoms during initiation. In study infants younger than age 6 months, encainide was associated with excessive QRS aberrancy during initiation in 4 of 13 (31%), compared with 3 of 28 (11%) in older children. Ventricular proarrhythmia occurred in 2 children and 1 died suddenly. Mean effective encainide dose was 3.5 mg/kg/day or 86 mg/m2/day. In 4 children who had nonextensive metabolism of encainide, the drug was ineffective. Encainide is effective in the treatment of some resistant forms of permanent junctional reciprocating tachycardia and AVRT in otherwise healthy children. Children younger than age 6 months and those with either previous proarrhythmic events or severe cardiac dysfunction appear to have a high incidence of adverse effects.
beta transgenic mice have a T-cell receptor beta-chain gene that is prematurely expressed on the surface of CD4- CD8- thymocytes and paired with an uncharacterized non-T-cell receptor alpha-chain polypeptide. The rearrangement of the T-cell receptor variable region gamma chain gene segment V gamma 4, a component of the gamma-chain gene that is rearranged and expressed preferentially on thymocytes of normal adult mice, is severely repressed in beta transgenic mice. Consequently no gamma delta T-cell receptor heterodimers are detectable on the surface of adult thymocytes or splenic T cells. These results indicate that cells expressing alpha beta or gamma (V gamma 4)-delta TCRs originate from a common precursor in which the first productive rearrangement of either the beta or gamma locus determines the further differentiation pathway into either alpha beta or gamma delta T cells. The repression of V gamma 4 rearrangement by a preexisting beta-chain gene may be indicative of one of several mechanisms which ensure that gamma delta and alpha beta receptors do not as a rule appear on the surface of the same cell.
We obtained electroretinograms (ERGs) from normal subjects and from patients with sickle cell disease. The ERG components (a-wave, b-wave, and oscillatory potentials) obtained from normal subjects and patients without peripheral retinal neovascularization did not differ in either amplitude or implicit time. However, ERG components obtained from patients with peripheral retinal neovascularization were reduced in amplitude compared with those obtained from normal subjects or patients without neovascularization. The reduced a-wave, b-wave, and oscillatory potential amplitudes may have been due to photoreceptor dysfunction secondary to choroidal ischemia or possibly increased oxygen demands by the inner retina. Ischemia of the inner retina may also have contributed to the altered b-wave and oscillatory potentials. These results suggest that ERG provides a means of assessing the consequence of peripheral retinal ischemia to retinal cell function and could be of value in monitoring patients with sickle cell disease for the development of clinically significant peripheral retinal neovascularization.
Our experience with the use of five new antiarrhythmic drugs for treating life-threatening arrhythmias in children will be briefly reviewed. Prevention of recurrent episodes of atrial flutter with digoxin and local anesthetic antiarrhythmic drugs often is only moderately successful, benefiting 65% of patients. Amiodarone is particularly useful for those patients who cannot be controlled on this regimen. We caution that the heart rate be monitored carefully when therapy with amiodarone is initiated in patients likely to have sick sinus syndrome. We have found mexiletine useful for controlling significant ventricular arrhythmias in patients with congenital heart disease. Likewise, 79% (11 of 14) of patients with ventricular tachycardia treated with amiodarone were well controlled. However, the range of disease categories (congenital heart disease, myocarditis, cardiomyopathy) in which amiodarone is effective is much broader than for mexiletine. Although other investigators have used amiodarone successfully for controlling supraventricular tachycardia in the Wolff-Parkinson-White syndrome or secondary to concealed accessory AV connections, we recommend surgical ablation. Propafenone has significantly improved our ability to control postoperative JET. Although JET is self-limited in duration and spontaneously remits, it frequently produces life-threatening hemodynamic compromise in the postoperative setting. Propafenone slows the ventricular rate into a range in which AV sequential pacing may be instituted. Generally, after 24 to 72 hours, the patient may be quickly weaned from propafenone. Chronic incessant supraventricular tachycardia (SVT) is frequently associated with a dilated cardiomyopathy. The two most common mechanisms of incessant SVT are PJRT and AET. We have found encainide and ethmozine extremely effective in suppressing tachycardia episodes in PJRT and AET, respectively. Medical therapy has been associated with few side effects.
A method of curing light-cured composite resins by transillumination to cement acid-etched fixed partial dentures was adapted to bond solid mesh-backed lingual orthodontic brackets. Results of this investigation showed that the bond strengths of the orthodontic brackets bonded with light-cured composite resins were significantly less (P less than 0.05) than the bond strengths of the orthodontic brackets cemented with traditional adhesives and orthodontic composite resins. Notwithstanding, the bond strengths achieved with the transilluminated light-cured composite resins should be adequate to withstand the forces of mastication and orthodontic movements. There was no correlation of bond strengths of the brackets cemented with the transilluminated light-cured composite resins when compared to the faciolingual widths of the teeth.
We investigated the retinal toxicity of various combinations of four antiviral agents in intravitreal injection and vitrectomy infusion solutions in the rabbit. The nontoxic concentration of each individual agent for intravitreal injection and for vitrectomy infusion has previously been described. Our results support the concept that concentrations of 200 micrograms/0.1 mL of trifluridine, 400 micrograms/0.1 mL of hydroxyacyclovir, 200 micrograms/0.1 mL of acyclovir and 30 micrograms/0.1 mL of vidarabine can be combined for intravitreal injection without toxic retinal effects. Moreover, 60 micrograms/mL of trifluridine, 20 micrograms/mL of hydroxyacyclovir, 40 micrograms/mL of acyclovir and 8 micrograms/mL of vidarabine can be combined in vitrectomy infusion solutions without retinal damage. Further study is needed to determine the full potential of such combinations.
Fifteen children, aged 5 days to 19 years (mean 4.7 years), with medically refractory supraventricular tachycardia were given oral encainide. In 10 of 15 children with "incessant" tachycardia (greater than 10% of the day), encainide alone controlled supraventricular tachycardia in 5 children; in combination with other antiarrhythmic agents, it partially controlled supraventricular tachycardia in 4 and was ineffective in 1. In 5 children with accessory atrioventricular connections, encainide eliminated supraventricular tachycardia in 3 and was ineffective in 2. Therapeutic encainide dosages ranged from 60 to 120 mg/m2/day (mean 90) (2.0 to 5.7 mg/kg/day). Encainide caused prolongation of the PR interval by 35%, RP interval by 17%, QRS interval by 44% and corrected QT interval by 10%. In 5 children with depressed left ventricular function administration of encainide, by controlling the arrhythmia, increased echocardiographic left ventricular shortening fraction from a mean of 24% to a mean of 36%. Three patients developed excessive QRS aberrancy, which was associated with wide QRS tachycardia in 2. No adverse reactions were noted in the absence of QRS aberration. Side effects were minor and noted in only 1 of 9 patients continuing to take the drug in 9 months of follow-up. Encainide was effective, or partially effective, in the control of resistant or incessant supraventricular tachycardia in 80% of children treated. Encainide allowed rapid resolution of arrhythmia-induced cardiomyopathy by controlling chronic supraventricular tachycardia.
The utility of the Abbreviated Injury Scale (AIS), the most widely used anatomic scale for rating severity of injuries, and its derivative for assessing the combined effect of multiple injuries, the Injury Severity Score (ISS), were tested for their ability to predict functional disability at time of discharge from the hospital and 6 months after discharge. The ISS has been shown to correlate well with mortality and length of stay, but the relationship to levels of subsequent disability has not been examined. Five hundred and ninety-seven patients (aged 16-45 years) were interviewed at time of discharge and 6 months after discharge to ascertain functional disability along three dimensions: activities of daily living (ADL), instrumental activities of daily living (IADL), and mobility. The authors report on the relationship between severity and functional disability at time of discharge and 6 months after discharge for a subset of 473 patients who did not suffer a severe brain injury. The results show that the relationship between ISS and status at discharge and 6 months after discharge is not monotonically increasing, as it is with mortality and length of stay (LOS). Rather, the proportion of people with severe injuries who report limitations is lower than for those with moderately severe injuries as defined by the ISS. Further, it is shown that the AIS of the most severe extremity and spinal cord injury carry considerably more weight when predicting functional status at discharge and 6 months after discharge than do the AIS scores of injuries to any other body region, although the relative explanatory power of each type of injury varies with the nature of the functional disability and the time interval between the initial insult and assessment.
The purpose of this study was to evaluate the use of exercise testing in identifying abnormalities of pacemaker function and in confirming set parameters not apparent at rest in children with implanted atrial synchronous physiologic pacemakers. Maximal exercise tests were performed on 24 children (15 boys, 9 girls) from 4.5 to 18 years of age (median = 15) with physiologic pacemakers. The lower rate limit was observed before or following testing in 19 of 24 cases. In each case this correlated with the set lower rate limit. The upper rate limit was reached in 10 of 24 cases and was found to be lower than that programmed in one case in which a long atrial refractory period had limited the upper rate limit. Six children reached the maximum upper rate limit to which their pacemaker could be programmed. No abnormalities of atrial capture or ventricular capture occurred during exercise testing. Ventricular sensing was normal in each case. Atrial sensing was observed to be normal in 15 of the 24 cases. Two patients had decreased atrial sensing with exercise. Reversion to the "noise rate" due to myopotential inhibition was found in seven other cases. Subsequent tests on two of these children showed normal sensing.(ABSTRACT TRUNCATED AT 250 WORDS)
Actively induced resistance to Eimeria ferrisi was studied clinically and histologically in Mus musculus. Results indicated that a partial resistance to challenge was obtained. Initially infected animals had severe symptoms of coccidiosis but the symptoms diminished as the infections progressed. Previously infected mice had only slight symptoms of infection when challenged. Challenge doses produced severe coccidiosis in the non-immune controls although none died. Six mice died at peak patency during the immunizing inoculations. Histologic examination indicated that parasite numbers were reduced in resistant animals. The reduction appeared greatest during late sexual development. Tissues from resistant animals showed little evidence of damage and appeared to contain an increased amount of lymphoid tissue. Tissues from non-immunized mice killed after challenge were heavily infected and contained extruded blood, mucous, and cellular debris in the gut lumen. Considerable destruction of host epithelial cells occurred. The observations are discussed and compared to other coccidial immunity studies.