Implicit processing and learning of visual stimuli in parietal extinction and neglect.
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Aged↗
Biomedical subjects
S Schwartz
Publications and source records attributed to S Schwartz.
Distinct patterns of word retrieval in right and left frontal lobe patients: a multidimensional perspective.
Several studies have shown that patients with frontal lobe damage (FL) generate fewer words than control participants in verbal fluency tasks. In the current study, we were interested in assessing the quality of such verbal fluency data. Multidimensional exploratory techniques, namely, hierarchical clustering (HC) and correspondence analysis (CoA), were performed on verbal fluency data collected from 13 FL patients and 11 age-matched controls. HC of individual word-profiles distinguished between patients with left frontal lobe (LFL) and right frontal lobe (RFL) lesions. CoA revealed that LFL patients generated unusual word-profiles, as evidenced by their peripheral location on a CoA map, while RFL patients produced highly typical word-profiles. We would like to suggest that preserved functioning of the left frontal lobe might have constrained the responses of the RFL group resulting in typical word combinations, whereas preserved right frontal lobe functioning might have mediated greater diversification in the responses of the LFL group. The present study shows that HC and CoA can be applied to neuropsychological data to reveal complex relationships between cognitive performance and cerebral lesion sites.
Aged↗
Heterogeneous nuclear ribonucleoprotein C binds exclusively to the functionally important UUUUU-motifs in the human papillomavirus type-1 AU-rich inhibitory element.
We have previously identified an inhibitory, 57 nt AU-rich sequence in the HPV-1 late 3' UTR, termed as the HPV-1 AU-rich element (h1ARE). It contains two types of functionally important motifs: two AUUUA sequences and three UUUUU sequences. We have shown that the h1ARE interacts with heterogeneous nuclear ribonucleoprotein (hnRNP) C1/C2 and the ELAV-like HuR protein. While we have shown that HuR binds to both the AUUUA- and the UUUUU-motifs, the interaction between hnRNP C and the h1ARE has not been investigated in detail. Here, we have used recombinant (r)hnRNP C1 to study the interaction between hnRNP C1 and the h1ARE by using the UV cross-linking assay. We demonstrate that (r)hnRNP C1 cross-links specifically to the three functionally important UUUUU-motifs in the h1ARE. In contrast, (r)hnRNP does not UV cross-link to the functionally important AUUUA-motifs in the h1ARE. Conclusively, the binding ability of hnRNP C to the h1ARE correlates with its partially inhibitory function. Additionally, the recombinant AU-rich RNA binding factor 1 (AUF1) was analyzed for binding to the h1ARE by using the UV cross-linking assay, but the results revealed no specificity for the functionally important AUUUA- and UUUUU-motifs.
3' Untranslated Regions↗
Determinants of information technology outsourcing among health maintenance organizations.
We analyze the determinants of HMO information technology outsourcing using two studies. Study 1 examines the effect of asset specificity on outsourcing for development and operation activities, using HMO specific fixed effects to control for differences between HMOs. Study 2 regresses the HMO specific fixed effects from Study 1, which measure an HMO's propensity to outsource, on HMO characteristics. The data comes from a 1995 InterStudy survey about information technology organization of HMOs. While HMOs split roughly equally in outsourcing information technology development activities, they are extremely unlikely to outsource the day-to-day operation of information systems. The greater an HMO's information technology capability and the complexity of information systems supported, the less likely is an HMO to outsource. While HMOs less than two years old, for-profit HMOs, local or Blue Cross-affiliated HMOs, and mixed HMOs are more likely to outsource, federally qualified HMOs are less likely to outsource. Policy and managerial implications for the adoption and diffusion of new ways of organizing information technology, such as application service providers (ASPs), are discussed.
Decision Making, Organizational↗
Effect of patch type on the cumulative irritation potential of 4 test materials.
BACKGROUND: Many different patch systems are available for predicting contact dermatitis. It is important to determine the ideal patch to meet the objective of the testing method. OBJECTIVE: The 21-day cumulative irritation test is well accepted for predicting irritation after repeated exposures. The patch type must allow separation of materials to predict irritation potential in the marketplace. Three patch systems were compared to determine which best provides this separation and prediction. METHODS: Four test materials were evaluated using 3 patch systems in a 21-day cumulative irritation test. Tested were water, 0.06% sodium lauryl sulfate (SLS), and 2 underarm products (UAP), one having lower and one having higher irritation potential. The patch types were; Webril pad and 8-mm and 12-mm Finn Chambers. RESULTS: Both the 12-mm Finn Chamber and Webril pad showed the ability to differentiate the higher irritating UAP and the 0.06% SLS from the lower irritation UAP product and water. The 8-mm Finn chamber was less discriminating, showing the 0.06% SLS to be the same as water and the lower-irritating UAP. CONCLUSION: The Webril pad and the 12-mm Finn Chamber are better at discriminating irritation potential than is the 8-mm Finn Chamber. The 12-mm Finn Chamber might also allow discrimination with a lower degree of irritation.
Adolescent↗
Physiological influences on off-finger glucose testing.
Products for monitoring blood glucose that allow extraction from sites other than the finger have recently been introduced. The FreeStyle Blood Glucose Monitor requires only 0.3 microL of blood, and allows extraction from the hand, arm, and leg, as well as the traditional finger site. Differences in circulatory physiology of the off-finger test sites lead to differences in the measured blood glucose concentration. The first study involved 160 clinic visits by 120 unique subjects with type 1 or type 2 diabetes. FreeStyle measurements were compared to YSI Model 2300 Stat Plus Glucose Analyzer plasma measurements using venous blood, capillary blood from the finger, and capillary blood from the arm. In a second study, the time course of glucose variation was tested by simultaneous measurements on the arm and finger taken every 15 min for 6 h. Thirteen subjects with type 1 diabetes were studied in two 6-h sessions. When FreeStyle was compared to YSI using venous samples and finger samples, the regression statistics were very similar. But when FreeStyle with arm samples was compared to YSI with finger samples, the regression equation was similar, but the scatter in the data was statistically significantly greater at the 95% confidence interval. By studying the time course of glucose changes, the difference between finger and arm measurements was attributed to a time lag in the glucose response on the arm with respect to glucose response on the finger. The lag was observed when the glucose concentration was increasing or decreasing, and the lag time varied from subject-to-subject in the range of 5-20 min. Using the Clarke Error Grid Analysis, the difference between arm and finger glucose measurements was not clinically significant. However, when the glucose concentration is decreasing rapidly into a state of hypoglycemia, the lag in measurements on the arm could delay detection of hypoglycemia. When specifically testing for hypoglycemia, the finger may be the preferable test site.
Arm↗
Functional neuroanatomical correlates of hysterical sensorimotor loss.
Hysterical conversion disorders refer to functional neurological deficits such as paralysis, anaesthesia or blindness not caused by organic damage but associated with emotional "psychogenic" disturbances. Symptoms are not intentionally feigned by the patients whose handicap often outweighs possible short-term gains. Neural concomitants of their altered experience of sensation and volition are still not known. We assessed brain functional activation in seven patients with unilateral hysterical sensorimotor loss during passive vibratory stimulation of both hands, when their deficit was present and 2-4 months later when they had recovered. Single photon emission computerized tomography using (99m)Tc-ECD revealed a consistent decrease of regional cerebral blood flow in the thalamus and basal ganglia contralateral to the deficit. Independent parametric mapping and principal component statistical analyses converged to show that such subcortical asymmetries were present in each subject. Importantly, contralateral basal ganglia and thalamic hypoactivation resolved after recovery. Furthermore, lower activation in contralateral caudate during hysterical conversion symptoms predicted poor recovery at follow-up. These results suggest that hysterical conversion deficits may entail a functional disorder in striatothalamocortical circuits controlling sensorimotor function and voluntary motor behaviour. Basal ganglia, especially the caudate nucleus, might be particularly well situated to modulate motor processes based on emotional and situational cues from the limbic system. Remarkably, the same subcortical premotor circuits are also involved in unilateral motor neglect after organic neurological damage, where voluntary limb use may fail despite a lack of true paralysis and intact primary sensorimotor pathways. These findings provide novel constraints for a modern psychobiological theory of hysteria.
Adolescent↗
Commentary: causes of incidence and causes of cases--a Durkheimian perspective on Rose.
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Causality↗
Image-guided transorbital roof craniotomy via a suprabrow approach: a surgical series of 72 patients.
OBJECTIVE: Many subfrontal and orbitofrontal craniotomy techniques have been developed. We present our results with the transorbital roof craniotomy, a frontal craniotomy that incorporates the orbital roof and is performed via a suprabrow incision. This technique was used in 72 patients, primarily for tumor resection. METHODS: Charts were retrospectively reviewed for all patients undergoing transorbital procedures. A total of 72 patients underwent 82 transorbital craniotomies from September 1995 to July 1999. The primary indication for the transorbital approach was mass lesion of the orbit, anterior fossa, or parasellar region. RESULTS: A total of 47 women and 25 men with a mean age of 53 years underwent 82 procedures. The primary pathological finding was meningioma, which occurred in 40 patients (55.6%), followed by craniopharyngioma (6.9%), pituitary macroadenoma (6.9%), schwannoma (5.5%), and hemangioma (5.5%). Simpson Grade I or II resection was achieved in 54% of patients, with Simpson Grade III to V resection achieved in the remaining 46%. Forty-one patients presented with visual loss in 43 cases, with 44.2% experiencing postoperative visual improvement, 46.5% remaining unchanged, and 9.3% worsening. Overall morbidity was 18.4%, with cerebrospinal fluid leak being the most common complication (6.6%). No patients died. CONCLUSION: The transorbital roof craniotomy is an evolutionary approach that provides excellent exposure to the orbit, anterior fossa, and parasellar region with little significant morbidity and, in our series, no mortality. Although we have used this approach primarily for resection of mass lesions, future directions for this procedure will likely lie in treating vascular lesions and lesions of the interpeduncular fossa.
Adult↗
The changing purpose of Prader-Willi syndrome clinical diagnostic criteria and proposed revised criteria.
BACKGROUND: Prader-Willi syndrome (PWS) is a complex, multisystem disorder. Its major clinical features include neonatal hypotonia, developmental delay, short stature, behavioral abnormalities, childhood-onset obesity, hypothalamic hypogonadism, and characteristic appearance. The genetic basis of PWS is also complex. It is caused by absence of expression of the paternally active genes in the PWS critical region on 15q11-q13. In approximately 70% of cases this is the result of deletion of this region from the paternal chromosome 15. In approximately 28%, it is attributable to maternal uniparental disomy (UPD; inheritance of 2 copies of a chromosome from the mother and no copies from the father, as opposed to the normal 1 copy from each parent) of chromosome 15, and in <2%, it is the result of a mutation, deletion, or other defect in the imprinting center. Clinical diagnostic criteria were established by consensus in 1993. Subsequently, definitive molecular genetic testing became available for laboratory diagnosis of PWS. However, identification of appropriate patients for testing remains a challenge for most practitioners because many features of the disorder are nonspecific and others can be subtle or evolve over time. For example, hypotonic infants who are still in the failure to thrive phase of the disorder often do not have sufficient features for recognition of PWS and often are not tested. Initial screening with these diagnostic criteria can increase the yield of molecular testing for older children and adults with nonspecific obesity and mental retardation. Therefore, the purpose of clinical diagnostic criteria has shifted from assisting in making the definitive diagnosis to raising diagnostic suspicion, thereby prompting testing. We conducted a retrospective review of patients with PWS confirmed with genetic testing to assess the validity and sensitivity of clinical diagnostic criteria published before the widespread availability of testing for all affected patients and recommend revised clinical criteria. METHODS: Charts of all 90 patients with laboratory-confirmed PWS were reviewed. For each patient, the presence or absence of the major, minor, and supportive features listed in the published diagnostic criteria was recorded. The sensitivity of each criterion, mean of the total number of major and minor criteria, and mean total score for each patient were calculated. RESULTS: There were 68 patients with a deletion (del 15q11-q13), 21 with maternal UPD of chromosome 15, and 1 with a presumed imprinting defect. Age range at the time of the most recent evaluation was 5 months to 60 years (median: 14.5 years; del median: 14 years; range: 5 months-60 years; UPD median: 18 years; range: 5-42 years). The sensitivities of the major criteria ranged from 49% (characteristic facial features) to 98% (developmental delay). Global developmental delay and neonatal hypotonia were the 2 most consistently positive major criteria and were positive in >97% of the patients. Feeding problems in infancy, excessive weight gain after 1 year, hypogonadism, and hyperphagia were all present in 93% or more of patients. Sensitivities of the minor criteria ranged form 37% (sleep disturbance and apneas) to 93% (speech and articulation defects). Interestingly, the sensitivities of 8 of the minor criteria were higher than the sensitivity of characteristic facial features, which is a major criterion. Fifteen out of 90 patients with molecular diagnosis did not meet the clinical diagnostic criteria retrospectively. CONCLUSION: When definitive diagnostic testing is not available, as was the case for PWS when the 1993 criteria were developed, diagnostic criteria are important to avoid overdiagnosis and to ensure that diagnostic test development is performed on appropriate samples. When diagnostic testing is available, as is now the case for PWS, diagnostic criteria should serve to raise diagnostic suspicion, ensure that all appropriate people are tested, and avoid the expense of testing unnecessarily. Our results indicate that the sensitivities of most of the published criteria are acceptable. However, 16.7% of patients with molecular diagnosis did not meet the 1993 clinical diagnostic criteria retrospectively, suggesting that the published criteria may be too exclusive. A less strict scoring system may ensure that all appropriate people are tested. Accordingly, we suggest revised clinical criteria to help identify the appropriate patients for DNA testing for PWS. The suggested age groupings are based on characteristic phases of the natural history of PWS. Some of the features (eg, neonatal hypotonia, feeding problems in infancy) serve to diagnose the syndrome in the first few years of life, whereas others (eg, excessive eating) are useful during early childhood. Similarly, hypogonadism is most useful during and after adolescence. Some of the features like neonatal hypotonia and infantile feeding problems are less likely to be missed, whereas others such as characteristic facial features and hypogonadism (especially in prepubertal females) may require more careful and/or expert examination. The issue of who should have diagnostic testing is distinct from the determination of features among confirmed patients. Based on the sensitivities of the published criteria and our experience, we suggest testing all newborns/infants with otherwise unexplained hypotonia with poor suck. For children between 2 and 6 years of age, we consider hypotonia with history of poor suck associated with global developmental delay sufficient criteria to prompt testing. Between 6 and 12 years of age, we suggest testing those with hypotonia (or history of hypotonia with poor suck), global developmental delay, and excessive eating with central obesity (if uncontrolled). At the ages of 13 years and above, we recommend testing patients with cognitive impairment, excessive eating with central obesity (if uncontrolled), and hypogonadotropic hypogonadism and/or typical behavior problems (including temper tantrums and obsessive-compulsive features). Thus, we propose a lower threshold to prompt diagnostic DNA testing, leading to a higher likelihood of diagnosis of this disorder in which anticipatory guidance and intervention can significantly influence outcome.
Adolescent↗
MDR-1 expression and deletions of chromosomes 7 and 5(Q) separately indicate adverse prognosis in AML.
In order to assess any correlation between MDR-1 expression and chromosomal aberrations, and to define their impact on clinical outcome in newly diagnosed AML pts, we investigated bone marrow and peripheral blood samples of 49 consecutive pts admitted to our hospital. Monosomy 7, trisomy 8 and 5q- were evaluated by means of interphase fluorescence in situ hybridization (FISH). Monosomy 7 was present in 6 pts, trisomy 8 in 5 pts, and 5q- in 6 pts. More than one aberration was seen in 7 pts. Chromosomal aberrations were mostly found in older pts (12/14 >60 years; p=0.03) and in pts with CD34 positive leukemic blasts (13/14 coexpressed CD34, p=0.0004). In 25 pts also standard G-banding analysis was performed leading to concordant results regarding chromosomes 7, 8 and 5. Flow cytometry identifyed MDR-1 positivity (MDR+) in 16 pts. MDR-1 expression appeared to be a characteristic feature in CD34+ AML (12/16 were CD34+ and MDR+ pts; p=0.013). No correlation, however, was found between chromosomal aberrations and MDR-1 expression. Pts with aberrations of either chromosomes 7, 8 or 5 detected by FISH (FISH+) were predominantly resistant to induction therapy (6/8 pts, p=0.004). A lower rate of complete remission (CR) was also seen in pts with MDR-1 expression (p=0.006). MDR+/FISH+ pts (n=3) were all refractory to remission induction, while all MDR-/FISH- pts (n=19) achieved CR (p=0.0006). MDR-1+ as well as pts with aberrations of chromosomes 7, and 5(q) showed a significantly decreased probability of overall survival. In conclusion, MDR-1 expression as well as abnormalities of chromosomes 7, and 5(q) predict poor clinical outcome in AML. The identification of these prognostic factors provides useful information for risk adapted treatment strategies.
Adult↗
[Conjunctival in situ carcinoma in a patient with Waldenström's disease].
A 73-year-old male patient was treated for conjunctival in situ carcinoma invading the cornea of his right eye. The patient had been previously operated on for two corneoconjunctival lesions on the same eye (one was a pterygium, the other was simple epithelial hyperplasia) and was regularly followed for a systemic lymphoplasmocytic lymphoma (Waldenström's disease). After a corneoconjunctival excision of the tumor, the histological analysis was performed and established the diagnosis of in situ carcinoma. The tumor recurred a few months later and radiation therapy was then given. No recurrence was observed after this latter treatment.
Aged↗
[Prophylaxis of infection in neutropenic patients. Guidelines of the Working Party on Infections in Hematology and Oncology].
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Antibiotic Prophylaxis↗
Uncoupling protein-1 mRNA expression in lipomas from patients bearing pathogenic mitochondrial DNA mutations.
Multiple symmetric lipomatosis (MSL) is a rare disorder characterised by large subcutaneous fat masses in some parts of the trunk. Mitochondrial disfunction is common in MSL, but the identity of the adipose cells developing in multiple lipomas is not well known. We determined that brown adipose tissue-specific uncoupling protein-1 (UCP-1) mRNA is expressed in the lipoma of a multiple symmetric lipomatosis patient bearing the 8344 mutation in the tRNALys gene of mitochondrial DNA. UCP1 mRNA was not detected in normal subcutaneous fat from the same patient or in the lipoma of another patient bearing a different mutation in the same tRNALys gene. These findings implicate brown adipose cells as the origin of lipomas in a subset of patients bearing tRNALys mutations in mitochondrial DNA.
Adipose Tissue, Brown↗
Betaine-homocysteine methyltransferase-2: cDNA cloning, gene sequence, physical mapping, and expression of the human and mouse genes.
Anomalies in folate and homocysteine metabolism can result in homocysteinemia and are implicated in disorders ranging from vascular disease to neural tube defects. Two enzymes are known to methylate homocysteine, vitamin B(12)-dependent methionine synthase (MTR) and betaine-homocysteine methyltransferase (BHMT). BHMT uses betaine, an intermediate of choline oxidation, as a methyl donor and is expressed primarily in the liver and kidney. We report the discovery of a novel betaine-homocysteine methyltransferase gene in humans and mice. The human BHMT2 gene is predicted to encode a 363-amino-acid protein (40.3 kDa) that shows 73% amino acid identity to BHMT. The BHMT2 transcript in humans is most abundant in adult liver and kidney and is found at reduced levels in the brain, heart, and skeletal muscle. The mouse Bhmt2 gene shows 69% amino acid identity and 79% similarity to the mouse Bhmt gene and 82% amino acid identity and 87% similarity to the human BHMT2 gene. Bhmt2 is expressed in fetal heart, lung, liver, kidney and eye. The discovery of a third gene with putative homocysteine methyltransferase activity is important for understanding the biochemical balance in using methyltetrahydrofolate and betaine as methyl donors as well as the metabolic flux between folate and choline metabolism in health and disease.
Amino Acid Sequence↗
Radiographic techniques for the pediatric patient.
Obtaining quality radiographs on pediatric patients can be a challenge. Suggestions for communicating with patients about radiation safety and the need for radiographs can facilitate the process. Guidelines for radiographic exposure intervals for young patients are key elements in the reduction of exposure to ionizing radiation and are presented in this paper. The child patient presents unique challenges for the dental professional and special techniques are presented in this article that may be helpful in the conducting radiographic examinations for this patient population.
Child↗
Adult with an interstitial deletion of chromosome 10 [del(10)(q25. 1q25.3)]: overlap with Coffin-Lowry syndrome.
We recently evaluated a mentally retarded 48 year old man found to have a cytogenetic deletion of chromosome 10 [46,XY,del(10) (q25. 1q25.3)]. Of interest, he shares many clinical findings with those described in Coffin-Lowry syndrome (CLS). These include severe mental retardation, short stature and a coarse facial appearance with widely spaced eyes, and patulous lips. He also had an extra transverse hypothenar crease, a finding that is seen in CLS. Furthermore, he has characteristic radiographic hand findings described in 95% of patients with CLS. The CLS gene, located at Xp22. 2, has recently been identified, and mutations in the Rsk-2 gene have been identified in several CLS patients. Rsk2 is part of a gene family implicated in cell cycle regulation through the mitogen-activated protein (MAP) kinase cascade. None of the currently recognized components of this pathway maps to the region deleted in our patient, nor are we able to identify any likely candidate genes in the deleted region, although several G protein coupled receptors have been cloned from the region. This patient's findings have some overlap with those seen in CLS, suggesting that a gene involved in MAP kinase signaling may be present in the deleted region of chromosome 10q25.1-25.3. Patients with a phenotype consistent with CLS, but lacking a family history suggestive of an X-linked disorder, should be evaluated with chromosome analysis paying particular attention to the region 10q25.
Abnormalities, Multiple↗
Efficacy and safety of recombinant human nerve growth factor in patients with diabetic polyneuropathy: A randomized controlled trial. rhNGF Clinical Investigator Group.
CONTEXT: Nerve growth factor is a neurotrophic factor that promotes the survival of small fiber sensory neurons and sympathetic neurons in the peripheral nervous system. Recombinant human nerve growth factor (rhNGF) has demonstrated efficacy as treatment for peripheral neuropathy in experimental models and phase 2 clinical trials. OBJECTIVE: To evaluate the efficacy and safety of a 12-month regimen of rhNGF in patients with diabetic polyneuropathy. DESIGN: Randomized, double-blind, placebo-controlled phase 3 trial conducted from July 1997 through May 1999. SETTING: Eighty-four outpatient centers throughout the United States. PATIENTS: A total of 1019 men and women aged 18 to 74 years with either type 1 or type 2 diabetes and a sensory polyneuropathy attributable to diabetes. INTERVENTIONS: Patients were randomly assigned to receive either rhNGF, 0.1 microg/kg (n = 504), or placebo (n = 515) by subcutaneous injection 3 times per week for 48 weeks. Patients were assessed at baseline, 12 weeks, 24 weeks, and 48 weeks. MAIN OUTCOME MEASURES: The primary outcome measure was a change in neuropathy between baseline and week 48, demonstrated by the Neuropathy Impairment Score for the Lower Limbs, compared between the 2 groups. Secondary outcome measures included quantitative sensory tests using the CASE IV System, the Neuropathy Symptom and Change questionnaire, the Patient Benefit Questionnaire (PBQ), and a global symptom assessment, as well as nerve conduction studies and occurrence of new plantar foot ulcers. Patients also were evaluated for presence of adverse events. RESULTS: Among patients who received rhNGF, 418 (83%) completed the regimen compared with 461 (90%) who received placebo. Administration of rhNGF was safe, with few adverse events attributed to treatment apart from injection site pain/hyperalgesia and other pain syndromes. However, neither the primary end point (P =.25) nor most of the secondary end points demonstrated a significant benefit of rhNGF. Exceptions were the global symptom assessment (P =.03) and 2 of 32 comparisons within the PBQ, which showed a modest but significant benefit of rhNGF (P =.05 for severity of pain in the legs and P =.003 for 6-month symptoms in the feet and legs). CONCLUSION: Unlike previous phase 2 trials, this phase 3 clinical trial failed to demonstrate a significant beneficial effect of rhNGF on diabetic polyneuropathy. JAMA. 2000;284:2215-2221.
Activities of Daily Living↗