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PubMed2004

Disorders of keratinization: diagnosis and management.

Disorders of cornification are a group of diseases that share abnormalities in the manufacture or desquamation of corneocytes. This paper reviews the major and a few of the rarer ones with a concentration on their therapy. Ichthyosis vulgaris is probably a post-translational defect in pro-filaggrin expression. It shows fine white flaky scales of the extensor surfaces, trunk, flank, lower legs but spares the folds and wet areas. Treatment is with aggressive moisturization. Hydrocortisone creams may be needed to control itch. Recessive X-linked ichthyosis is due to a deficiency of cholesterol sulfatase. Boys with this condition show small dark scales around the ears, sides of the neck, extensor surfaces of the arms and legs, and the peri-umbilical region. It spares the folds and face. Treatment is with moisturizers, topical retinoid creams or with topical cholesterol-based creams. Checking for signs of contiguous gene disorders (Kallman or Conradi-Hunermann syndromes) is necessary. Bullous congenital ichthyosiform erythroderma is caused by mutations in keratins 1 and/or 10. These patients are born as bright red babies with large blisters and erosions. Slowly, a porcupine quill-like waxy scaling develops. Blistering continues throughout life. Secondary infections of the skin cause pain, debility, and a very foul odor. Treatment is difficult. Topical moisturizers, descalers and retinoid creams help a little. Oral retinoids help a lot but can cause increased blistering. Controlling the odor is an ongoing issue using antibacterial washes, absorbing powders, and masking fragrances. Autosomal recessive ichthyosis is a term for both lamellar ichthyosis and congenital ishthysosiform erythroderma. They are caused by various mutations in transglutaminase-1 gene. In both instances patients are born as 'collodion babies'. Lamella ichthyosis has the very recognizable plate-like scale over the entire body. Children with congenital ishthysosiform erythroderma are red all over with a finer scale in some places and plate-like scales in others. Treatment is with topical moisturizers, retinoid creams, descalers, and in some cases oral retinoids. Palmar plantar keratodermas occur in conjunction with some ichthyoses, but also by themselves. Some are diffuse and others have discrete, corn-like hardenings. Treatment with topical acids, propylene glycol and retinoid creams help to some extent.Throughout the article pearls from my practice are included to assist the clinician in the day-to-day handling of these patients. A short section on genetic counseling concludes this article.

Filaggrin Proteins↗

PubMedDate not supplied

[Measurement of the quality of life in stroke survivors].

OBJECTIVE: The aim of this article is to describe methods of evaluation of the quality of life in relation to health after a stroke, methodological considerations and criteria for the use of scales. DEVELOPMENT: The terms affected person, disability and handicap are defined. We discuss the indices of functional evaluation used in the study of strokes: activities of daily life (ADL), instrumental activities and the generic health questionnaires used to study quality of life. Ideally, these should all be reliable, valid and sensitive to change. The profile of the consequences of the illness, the SF-36 and Nottingham health questionnaires are valid instruments for evaluation of the quality of life of people with strokes. The Barthel index is a reliable, valid index for measurement of ADL in patients with strokes. The Rankin scale is used to evaluate disability and handicap. The indicia of activity of these instruments involve a cultural bias due to their original design. The risk of falls, painful shoulders, the presence of dysphagia or of urinary incontinence are specific complications of stroke patients. Difficulty in returning to work or driving are factors affecting the quality of life after stroke. CONCLUSION: In the follow-up survivors with strokes it is necessary to include quality of life instruments for measurement of the dimensions of health not covered by ADL indices, especially social and emotional functions and functional state.

Activities of Daily Living↗

PubMed2005

Workstyle: development of a measure of response to work in those with upper extremity pain.

Workstyle or the behavioral, cognitive, and physiological response that can occur in some individuals to increases in work demands has been proposed to help explain the link between ergonomic and psychosocial factors in the exacerbation of work-related upper extremity symptoms. Currently, there is no measure of this construct, hindering research on its potential link to work related upper extremity problems in the workplace. The present study describes the development and psychometric properties of a measure of workstyle. Questionnaire items reflecting dimensions of workstyle as per the original conceptualization were generated primarily through focus groups with office workers and separate groups held with occupational physicians, physical therapists, occupational health psychologists, and experts in ergonomics, behavioral science, and human factors. Items created through this process were then administered to 282 symptomatic and asymptomatic office workers. Measures of job stress, ergonomic risk, upper extremity symptoms, and functional limitations were also obtained. The workstyle questionnaire was divided into two broad dimensions: Characteristic responses to work and Response to increased work demands. The scale development process as indicated by factor analysis yielded subscales that are theoretically consistent with the workstyle construct. These subscales include: working through pain, social reactivity at work, limited workplace support, deadlines/pressure, self imposed work pace/workload, breaks, mood, pain/tension, autonomic response, and numbness tingling. The internal consistency of these subscales varied from 0.61 to 0.91, n = 282 while the test-retest (3 weeks) reliability for the various subscales ranged from r = 0.68 to 0.89, n = 143. A total workstyle score was computed that excluded the pain/tension and numbness/tingling subscales to avoid circular reasoning in terms of the measure's relationship to outcomes of pain and functional limitations. The total score was stable over time and provided unique variance in relation to traditional measures of job stress. Total workstyle score was significantly associated with higher levels of pain, and greater functional limitations. Dimensions of the workstyle construct were identified. The workstyle measure possesses acceptable psychometric properties in office workers who work with computers. This measure can be used in future studies on the interaction of psychosocial and ergonomic factors in the exacerbation of upper extremity pain and functional limitation.

Adult↗

PubMed2004

Long-term behavior of cooling fluid in a rectangular container.

In this study, the long-term behavior of cooling an initially quiescent isothermal Newtonian fluid in a rectangular container with an infinite length by unsteady natural convection due to a fixed wall temperature has been investigated by scaling analysis and direct numerical simulation. Two specific cases are considered. Case 1 assumes that the cooling of the fluid is caused by the imposed fixed temperature on the vertical sidewall while the top and bottom boundaries are adiabatic. Case 2 assumes that the cooling is caused by the imposed fixed temperature on both the vertical sidewall and the bottom boundary while the top boundary is adiabatic. The appropriate parameters to represent the long-term behavior of the fluid cooling in the container are the transient average fluid temperature T(a)(t) over the whole volume of the container per unit length (i.e., the transient area average fluid temperature, as used in the subsequent numerical simulations) at time t and the average Nusselt number on the cooling boundary. A scaling analysis has been carried out which shows that for both cases theta(a)(tau) scales as e(-C(ARa)(-1/4) tau), where theta(a)(tau) is the dimensionless form of T(a)(t), tau is the dimensionless time, A is the aspect ratio of the container, Ra is the Rayleigh number, and C is a proportionality constant. A series of direct numerical simulations with the selected values of A, Ra, and Pr (Pr is the Prandtl number) in the ranges of 1/3< or =A< or =3, 6 x 10(6) < or =Ra< or =6 x 10(10), and 1< or =Pr< or =1000 have been carried out for both cases to validate the developed scaling relations. It is found that these numerical results agree well with the scaling relations. The numerical results have also been used to quantify the scaling relations and it is found that C=0.645 and 0.705 respectively for Cases 1 and 2 with Ra, A and Pr in the above-mentioned ranges.

Biophysics↗

PubMed2006

Normal neuropsychological development in children with congenital complete heart block who may or may not be exposed to high-dose dexamethasone in utero.

BACKGROUND: Antenatal and postnatal treatment with dexamethasone (DEX) may negatively affect the neuropsychological development in children. Maternal anti-Ro/Sjögren's syndrome A (SSA) antibodies may also be associated with learning disabilities in offspring. OBJECTIVE: To assess neuropsychological development in babies exposed to very high dosages of DEX in utero, whose mothers were anti-Ro/SSA positive. METHODS: 13 children with congenital complete heart block (CHB) (11 exposed and 2 not exposed to DEX) and 3 healthy siblings, all of anti-Ro/SSA-positive women, were evaluated. 11 preschool-aged children (5 boys) were assessed using Griffiths Mental Development Scales. 5 school-aged children (2 boys) were examined using Wechsler Intelligence Scale for Children-Revised to check IQ and reading tests to explore the existence of learning disabilities or dyslexia. None of the children had had major neonatal complications, although those with CHB had to be paced at different intervals from birth. RESULTS: The children had been exposed in utero to a mean total dose of 186.6 mg DEX. IQ levels were always normal (mean IQ 105.1, standard deviation (SD) 9.5). Only one child had a learning disability, of borderline clinical significance, but this child had never been exposed to DEX. CONCLUSION: No negative effects were found on the neuropsychological development in this cohort of children, even if they had been exposed to maternal anti-Ro/SSA antibodies and to very high dosages of DEX (much higher than those used to improve fetal lung maturity). These findings might be of interest in view of the large number of infants exposed in the past to repeated antenatal courses of steroids.

Antibodies, Antinuclear↗

PubMed2000

Carotid artery injuries and their management.

BACKGROUND: Major vascular injuries in the region of the neck are most frequently the result of penetrating trauma. Evaluation and management of patients with injury to Zone II of the neck remains highly controversial. Most studies involve small number of patients with a lack of standardization of the nature of the injury in reporting outcome. It is the purpose of this study to propose a grading scale for vascular injuries in the neck that would allow for more uniform reporting of such injuries. METHODS EXPERIMENTAL DESIGN: A retrospective review of all patients treated for penetrating trauma to the neck was performed and the subset of patients with major vascular injuries identified. Data from this group of patients are presented. SETTING: Level II urban trauma center. PATIENTS AND INTERVENTIONS: During the period July 1984 to June 1994, 107 patients were treated for penetrating neck trauma. Injuries to the major arteries of the neck were present in 18 of the 107 patients (16.8%). All injuries were graded on the developed scale. Management protocol was based on the grade of the injury. Grade 1 injuries were managed non-operatively with systemic anticoagulation and low molecular weight dextran. Grade 2 injuries were treated with primary repair. Injuries of Grades 3 and 4 were treated by primary repair or interposition graft. Exceptions were isolated injuries of the external carotid artery, which were treated by ligation alone. RESULTS: Of the 18 patients with carotid artery injuries, 2 had injuries of the external carotid artery, treated with ligation alone. The internal carotid artery was injured in 7 cases. An interposition saphenous vein/PTFE graft was used in all cases. In 9 cases the common carotid artery was injured. Repair was accomplished by a combination of either a primary repair or interposition graft. Overall mortality was 3/16 (16.6%). No new or worsening of neurologic deficit occurred in any patient. CONCLUSIONS: Carotid artery injuries occur in about 17% of patients with penetrating neck trauma. Data regarding management and prognosis in these patients are at best concflicting, in part, due to lack of a standardized classification system. The proposed grading scale is designed to overcome this problem.

Adolescent↗

PubMed2001

Body image, strategies to change muscles and weight, and puberty: do they impact on positive and negative affect among adolescent boys and girls?

The purpose of this study was to examine the impact of strategies to both decrease weight and increase muscle tone on negative (depression, anxiety) and positive affect among adolescent males and females. The respondents were 1185 adolescents (587 males, 598 females) who were enrolled in grades 7 and 9 (mean age for males=13.22 years; mean age for females=13.21 years). Respondents completed the Body Image and Body Change Inventory that assessed body image satisfaction, body image importance, body change strategies to decrease weight, body change strategies to increase muscle tone, and food supplements. Respondents also completed the Pubertal Development Scale, the depression and anxiety scales of the Depression Anxiety and Stress Scale, and the positive affect items from the Positive and Negative Affect Scale. Structural equation modelling was used to determine how body satisfaction and importance, body change strategies, and puberty impacted on depression, anxiety, and positive affect. The results demonstrated that for both boys and girls, there was a strong association between body change strategies and negative affect. For boys, body dissatisfaction did not predict negative affect, although this was a strong predictor for girls. Body change strategies did not strongly predict positive affect for either boys or girls, although body image satisfaction was a strong predictor for both genders. The implications of these findings for obtaining a better understanding of the role of pubertal development, body image, and body change strategies in predicting positive and negative affect among adolescent males and females are discussed.

Journal Article↗

PubMed2005

The menopausal transition: does it induce women's worries about aging?

OBJECTIVE: This study examines whether patterns of worries about aging change in women during the menopausal transition. DESIGN: A population-based sample of 261 Australian-born women aged 45-55 years at baseline participated in a longitudinal study. Data were collected at baseline and in the 5th year of annual follow-up interviews. Measures included the Attitudes to Aging Scale which is based on a scale developed by Kaufert and Syrotuik and contained the six-item version which examines worries about specific losses. Menopausal status was determined by menstrual status as follows: late reproductive, early menopausal transition, late menopausal transition, and postmenopause. RESULTS: Data analysis carried out by cross-tabulation found that 36% of participants did not change their attitudes over the 5-year follow-up period, 30% became more worried and 33% became less worried and there was no significant difference between those who had or had not experienced the menopausal transition. There was no significant association between the scores on the Attitudes to Aging Scale and changes in the self-rated health, work status and marital status. CONCLUSION: The menopausal transition does not induce an overall increase in worries about aging.

Aging↗

PubMed1995

Feminist identities and preferred strategies for advancing women's positive self-concept.

This study was an attempt to (a) determine the generalizability of the American-based Feminist Identity Development Scale (FIDS; Bargad & Hyde, 1991) to female students in a New Zealand university and (b) examine the relationship between feminist identities (as measured by the FIDS) and preferred strategies for advancing women's self-concept. Female undergraduate university students (N = 145) were given the FIDS and a self-concept strategy questionnaire. The factor structure of the FIDS was replicated for four of the five stages reported by Bargad and Hyde. Failure to replicate the remaining stage (revelation) was attributed to differences in the sample populations and the wording of the subscale. Positive correlations were found between the early stage of feminist identity development and individual-oriented self-concept strategies and also between the late stage of feminist identity development and group-oriented self-concept strategies. These results suggest that as a woman's feminist identity develops, the strategies for advancing that self-concept change from individual- to group-oriented ones.

Adolescent↗

PubMed2002

A fast empirical approach to binding free energy calculations based on protein interface information.

Three useful variables from the interfaces of 20 protein-protein complexes were investigated. These variables are the side-chain accessible number (N(b)), the number of hydrophilic pairs (N(pair)) and buried a polar solvent accessible surface areas (DeltaDeltaASA(apol)). An empirical model based on the three variables was developed to describe the free energy of protein associations. As the results show, the side-chain accessible numbers characterize the loss of side-chain conformational entropy of protein interactions and the effective empirical function presented here has great capability for estimating the binding free energy. It was found that the variables of interface information capture most of the significant features of protein-protein association. Also, we applied the model based on the variables as a rescoring function to docking simulations and found that it has the potential to distinguish the 'true' binding mode. It is clear that the simple and empirical scale developed here is an attractive target function for calculating binding free energy for various biological processes to rational protein design.

Algorithms↗

PubMed1983

Measures of autonomy.

Two studies examined the validity of 2 CPI autonomy scales developed by Kurtines (1973, 1978), In the first study, a criterion rating of autonomy based upon observer descriptions of adults (n = 314) was determined from the California Q-set. This 10-item criterion was validated in a second study with college sophomores (n = 142) Using the criterion with a combined sample. of subjects, individuals high or low on autonomy were identified. Personality profiles of each group were determined from their responses to the CPI. Personality dimensions associated with ascendancy, self-assurance, interpersonal adequacy, and achievement orientation differentiated the two criterion groups. Correlation and multiple regression analyses indicated that Kurtines' first (1973) autonomy scale by comparison .predicted these personality dimensions Substantially better than his second (1978) scale, It was concluded, however, that neither scale adequately measured the global personality construct Of autonomy because (a) each scale assessed autonomous rule compliance to the exclusion of autonomous rule defiance, and (b)each scale measured only socially desirable aspects of autonomy.

Journal Article↗

PubMed2000

Simulations of a meter-long plasma wakefield accelerator.

Full-scale particle-in-cell simulations of a meter-long plasma wakefield accelerator (PWFA) are presented in two dimensions. The results support the design of a current PWFA experiment in the nonlinear blowout regime where analytic solutions are intractable. A relativistic electron bunch excites a plasma wake that accelerates trailing particles at rates of several hundred MeV/m. A comparison is made of various simulation codes, and a parallel object-oriented code OSIRIS is used to model a full meter of acceleration. Excellent agreement is obtained between the simulations and analytic expressions for the transverse betatron oscillations of the beam. The simulations are used to develop scaling laws for designing future multi-GeV accelerator experiments.

Journal Article↗

PubMed2004

Influence of sleep position experience on ability of prone-sleeping infants to escape from asphyxiating microenvironments by changing head position.

OBJECTIVE: Several studies have found that back- or side-sleeping infants who are inexperienced in prone sleeping are at much higher risk for sudden infant death syndrome (SIDS) when they turn to prone or are placed prone for sleep compared with infants who normally sleep prone. Moreover, such inexperienced infants are more likely to be found in the face-down position at death after being placed prone compared with SIDS infants who are experienced in prone sleeping. We hypothesized that lack of experience in prone sleeping is associated with increased difficulty in changing head position to avoid an asphyxiating sleep environment. METHODS: We studied 38 healthy infants while they slept prone. Half of these were experienced and half were inexperienced in prone sleeping. To create a mildly asphyxiating microenvironment, we placed infants to sleep prone with their faces covered by soft bedding. We recorded inspired CO2 (CO2I), electrocardiogram, and respiration, and we videotaped head movements. Also, we assessed gross motor development (Denver Development Scale). RESULTS: When sleeping prone, with their faces covered by bedding, all infants experienced mild asphyxia as a result of rebreathing. All aroused and attempted escape from this environment. Infants used 3 stereotyped head-repositioning strategies. The least effective was nuzzling into the bedding with occasional brief head lifts. More effective were head lifts combined with a head turn. Some infants, however, could turn only to 1 side, right or left. Infants who were inexperienced in prone sleeping had less effective protective behaviors than experienced infants. Infant age did not correlate with efficacy of protective behaviors. Infants who were experienced in prone sleep had advanced gross motor development compared with inexperienced infants. CONCLUSION: Infants who are inexperienced in prone sleeping have decreased ability to escape from asphyxiating sleep environments when placed prone. These observations potentially explain the increased risk associated with prone sleep in infants who are inexperienced. The increased occurrence of the face-down position in such infants is also potentially explained. These findings suggest that airway protective behaviors may be acquired through the mechanism of operant conditioning (learning).

Aging↗

PubMed2002

Total body water measurement using bioelectrical impedance analysis, isotope dilution and total body potassium: a scoring system to facilitate intercomparison.

OBJECTIVES: (1) To develop a scale that is useful in evaluating the accuracy of multifrequency bioelectrical impedance analysis (MF-BIA) in the assessment of body water volumes against the accepted gold standard measurements based on isotope-dilution and total body potassium (TBK). (2) To perform a pilot test of the scale. DESIGN: A scale was developed to evaluate the accuracy of MF-BIA in the assessment of body water volumes. Questions were obtained from reading the scientific literature and discussions involving the four authors. Three of these and two additional independent readers pre-tested the scale. A weighting was identified for each question and a pilot test with a sample of 10 articles (different to those used for the questionnaire performance) was conducted. A further validation was carried out with a second set of 20 articles and two additional independent readers. RESULTS: The kappa statistic expressing the level of agreement between pairs of the first three authors using this scale with 10 articles, was 0.3, 0.4 and 0.6 after the first attempt. A second evaluation after specific changes improved the agreement to 0.8, 0.6 and 0.8. The mean score for 10 articles was 252+/-36 points from a total score of 400 (63+/-9%). The evaluation with the second set of 20 articles resulted in a kappa of 0.7 from two pairs of authors. The evaluation with two additional reviewers resulted in a kappa=0.7. CONCLUSION: A tool has been developed to assess the accuracy of the MF-BIA technique and to identify methodological components, plan future studies and critically evaluate data in this area. It is likely that this tool may also be used to assess the accuracy of single frequency studies.

Body Water↗

PubMed1992

Relationships among an older adult's life review, ego integrity, and death anxiety.

The frameworks of Erikson (1963) and Butler (1963) were used to design this descriptive study that investigated the relationships among life review, ego integrity, and death anxiety in older adults. Three hypotheses were proposed: (a) the greater the life review, the higher the ego integrity; (b) the greater the life review, the lower the death anxiety; (c) the higher the ego integrity, the lower the death anxiety. The sample consisted of 115 female and male volunteers between the ages of 65 and 93. The participants filled out the Life Review Questionnaire, Adult Ego Development Scale, Death Anxiety Scale, Death Preparation Scale, and a Personal Information Sheet. Life review, while not positively correlated with ego integrity, was found to have a negative correlation with death anxiety. Also, religious subjects were found to be more prepared for death than those who did not practice. Implications for therapy and future research are discussed, as well.

Aged↗

PubMed2005

Pre- and postoperative developmental attainment in sagittal synostosis.

AIMS: To investigate whether sagittal synostosis (SS) has consequences for children's mental and psychomotor development, and whether surgery has any impact on this. METHODS: The study involved 28 children with SS who underwent corrective surgery at a mean age of 8.0 (SD 7.16) months, and 28 normal controls. All the children with SS were assessed pre- and postoperatively using the Griffiths Mental Development Scales. The controls were assessed on one occasion, at an age matched with individuals in the patient group at the time of the preoperative assessment. A further control group consisted of 13 children with SS, who had received developmental assessment on two or more occasions without surgical intervention. RESULTS: The data indicated that children with SS have significantly poorer gross locomotor function than the normal controls. Following surgical intervention the deficit was shown to have resolved; consistent with this a lesser improvement in eye-hand coordination and performance skills was shown. Overall developmental attainment also improved postoperatively. The children with SS who did not receive surgery did not show any improvement in development. CONCLUSIONS: The study shows improved developmental attainment following surgical correction of SS, which may therefore be more than a cosmetic procedure.

Analysis of Variance↗

PubMed2006

[Study on tetrahydrobiopterin deficiency in Northern Chinese population].

OBJECTIVE: To emphasize early differential diagnosis from patients with hyperphenylalaninemia (HPA) and to evaluate the treatment and long-term outcome of patients with tetrahydrobiopterin synthase (BH4) deficiency in Northern Chinese population. METHODS: From 1992 to 2005, a total of 618 patients with HPA were diagnosed and/or cared for in our outpatient clinic. Urinary pterin analysis, detection of dihydropteridine reductase (DHPR) activity in blood, and then BH4 loading tests were carried out to differentiate BH4 deficiency in these patients from classical phenylketonuria. BH4 deficient patients were treated with BH4, levodopa and 5-hydroxytryptophane (5-HTP) immediately while the diagnosis was done to disease. Patientso blood phenylalanine levels, psychomotor and intelligence development were followed up. RESULTS: A total of 38 cases were diagnosed as BH4 deficiency, all of them were revealed as 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency from the extremely decreased urine biopterin, normal DHPR activities and drop down of blood phenylalanine level to normal range within 4 to 8 hours after BH4 loading. The most common manifestations were progressively psychomotor and mental retardation to patients even after taking early dietary treatment. The patients were diagnosed and treated with drugs at the ages of 2.1 months to 13 years. With 4 patients died of pneumonia, 7 patients refused to treatment, only 27 patients were under treatment and followed up. The average full scale development or intelligence quotient (DQ/IQ) of patients who were treated within and after 6 months were 86+/- 10 or 66+/- 7 respectively. Development was not even in different aspects. A significant negative correlation was observed between the level of the DQ and the age of treatment commenced (r was -0.714, P< 0.01). Eleven patients experienced the extrapyramidal movement disorders, 3 of them combined with epilepsy. The extrapyramidal disorders were controlled by administration of levodopa. CONCLUSION: The differential diagnosis for BH4 deficiency should be carried out in all patients with HPA. PTPS deficiency is the most common form of BH4 deficiency in Northern Chinese population. The long-term outcome of these patients benefits from diagnosis and treatment with BH4, levodopa and 5-HTP as early as possible.

5-Hydroxytryptophan↗

PubMed1988

Affinity purification of human plasma fibronectin on immobilized gelatin.

Several problems are associated with the biospecific affinity purification of plasma fibronectin on gelatin-Sepharose. Large-scale development of this purification procedure requires optimization of adsorption and elution conditions. The adsorption capacity depends on the amount of gelatin coupled to the Sepharose, the residence time, the temperature and the amount of fibronectin loaded on the adsorbent. Elution of adsorbed fibronectin with 3 M urea leads to incomplete recovery. The elution yield was found to vary with both the gelatin concentration and the amount of adsorbed fibronectin. Despite the incomplete elution, the adsorption capacity did not decrease after twelve consecutive isolation procedures. Under optimized conditions, the method described here provides a rapid, single-step and convenient way for the isolation of pure and functional fibronectin, either for analytical or large-scale preparative purposes.

Adsorption↗