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At least 91 records · Page 5Linked to original sources
PubMed1988

Intramedullary fixation of subtrochanteric fractures with the Williams Y-nail: report of three cases.

Küntscher Y-nailing is indicated for unstable proximal fractures of the femur in the elderly (5-7,10). The Williams modified Y-nail is an interlocking Y-nail that was designed to extend the indications beyond those of the Küntscher Y-nail. The Williams Y-nail was employed in three cases of subtrochanteric and subtrochanteric-intertrochanteric fractures, including one case in which there was an ipsilateral femoral shaft fracture. This procedure requires reaming and interlocking which are not performed in classic Küntscher Y-nailing. At follow-up (14-month minimum), each patient had a good clinical result and radiographic healing. Leg length shortening (maximum, 1.5 cm) occurred in two of three cases, primarily as a result of the failure to employ either a proximal or a distal interlocking screw. No other deformities have been observed. Williams Y-nailing can extend the indications of the Küntscher Y-nail to include rotationally unstable subtrochanteric fractures and associated ipsilateral femoral shaft fractures. The increased technical demands of the Williams modified Y-nail warrant cautious use depending upon the complexity of the fracture. Used properly, the Williams Y-nail may reduce the incidence of rotational and leg length deformities often experienced with other commonly used devices for unstable subtrochanteric and ipsilateral trochanteric-diaphyseal fractures of the femur.

Aged↗
PubMed2006

Clinical report: a case of Williams Syndrome and Klinefelter Syndrome.

INTRODUCTION: Williams syndrome (WS) is a rare but well recognised neurodevelopmental disease affecting the connective tissue and the central nervous system. Many patients are identified through the presence of dysmorphic features and associated cardiac abnormalities. Klinefelter syndrome (KS) is associated with gynaecomastia, small testes, azoospermia and elevated gonadotropin levels. They are recognised in the second decade of life by their tall stature and delay in pubertal development. A combination of constitutive WS and KS has yet to be described. CLINICAL PICTURE: We report a child with these genetic aberrations, highlighting the clinical characteristics of such an individual. CONCLUSION: The manifestations and interactions of both conditions are also discussed.

Body Height↗
PubMed1994

Cerebrovascular stenoses with cerebral infarction in a child with Williams syndrome.

We report on a patient with Williams syndrome who suffered a cerebrovascular accident. Clinical evaluation demonstrated the presence of carotid and cerebral arterial stenoses. We believe these lesions led to acute cerebrovascular ischemia and a non-hemorrhagic cerebral infarction. It is possible the stenoses were exacerbated by a vasculitis. The stenoses were identified by both invasive and noninvasive imaging studies. These studies may have a role in the evaluation of persons with Williams syndrome.

Abnormalities, Multiple↗
PubMed2005

Right atrial mass in a child with disseminated coccidioidomycosis.

A 10-year-old patient with known coccidioidomycosis relapsed and had dysrrhythmias and a right atrial mass. Histopathology and culture after surgical removal revealed that this was a vegetative mass infected with Coccidioides spp. We believe that this is the first case of coccidioidal endocarditis to be reported.

Amphotericin B↗
PubMed1990

Temperament in Williams syndrome.

Anecdotal reports suggest that children with Williams syndrome are loquacious, affectionate, charming, open, and gentle. The temperament, or behavioral style, of individuals with Williams syndrome was assessed using standard temperament scales of parental response. Children with Williams syndrome appear, on average, to display higher activity, lower rhythmicity, greater approach, lower adaptability, greater intensity, more negative mood, less persistence, greater distractibility, and lower threshold to arousal than will average children. Older children with Williams syndrome will likely be viewed as "difficult." Despite this overrepresentation in the "difficult" cluster, on average, this population differs from the usual population of "difficult" children in being approaching, rather than displaying a tendency to withdraw. Parents of a child with Williams syndrome should be apprised of potentially negative temperament characteristics, relieved of the burden of responsibility for having a difficult child who "should" be easy, and counselled, when appropriate, regarding the efficacy of modification of parent-child interactions with children having the difficult temperament.

Abnormalities, Multiple↗
PubMed2005

The binding of 1,8 ANS congeners to I-FABP and comparison of some hypotheses about ANS' spectral sensitivity to environment.

The ANS congeners 1-anilinonaphthalene and 1-amino,8-sulfonato naphthalene were investigated as analogs of 1,8 anilinonaphthalene sulfonate. Like 1,8 ANS, they also bind to I-FABP, and the fully-bound spectra reveal interesting similarities and differences with respect to ANS binding. The nature of these differences suggests that certain hypotheses in the literature about ANS photophysics ought to be revised. The conceptual decomposition of energetic effects in the thermodynamics of ANS binding proposed earlier [W. Kirk, E. Kurian, F. Prendergast, Characterization of the sources of protein-ligand affinity: 1-sulfonato-8-(1')anilinonaphthalene binding to intestinal fatty acid binding protein. Biophys. J. (1996) 70 69-83.] is extended further in this report.

Anilino Naphthalenesulfonates↗
PubMed2006

Application of the 1994 WHO classification to populations other than postmenopausal Caucasian women: the 2005 ISCD Official Positions.

In 2003, the International Society for Clinical Densitometry (ISCD) developed Official Positions regarding the applicability of the World Health Organization (WHO) classification of bone mineral density to populations other than postmenopausal women. However, these prior Official Positions do not fully address bone mineral density reporting in females prior to menopause, men, and non-whites. During the 2005 ISCD Position Development Conference, members of the ISCD Expert Panel in conjunction with the ISCD Scientific Advisory Committee re-addressed these topics and, based upon stringent reviews of best available data, developed ISCD Official Positions that provide greater specificity and clarification with respect to the following: (1) the utility of the term 'osteopenia'; (2) utilization of T- and Z-scores for bone mineral density reporting; (3) when to apply the WHO densitometric classification; and (4) which normative database(s) should be used for non-white individuals. Briefly, the term "osteopenia" is retained, but 'low bone mass' or 'low bone density' is preferred. Z-scores, not T-scores, are preferred in females prior to menopause and males under age 50. In these individuals, a Z-score of -2.0 or lower is defined as "below the expected range for age" and a Z-score above -2.0 is "within the expected range for age." T-scores are preferred and the WHO classification is applicable for postmenopausal women and men age 50 and older. These Official Positions, rationale and evidence are discussed in the following report.

Absorptiometry, Photon↗
PubMed2002

Child custody evaluations.

Because divorce is so common, it is important for our society to find ways to minimize the psychological trauma that is experienced by children of divorced parents. Ideally, divorcing parents would not fight so much over the children, in front of the children, and through the children. When disputes do arise regarding custody, visitation, and parenting plans, mental health professionals can assist the judges who make the final decisions by performing competent custody evaluations. These evaluations should be conducted in a systematic manner, should consider several critical factors in an unbiased manner, and should result in recommendations that promote the best interests of the children. In most cases, the goal is for each child to have strong, healthy relationships with both parents. After conducting an evaluation, it is usually possible to make recommendations regarding custody, parenting arrangements, and forms of counseling and therapy that should be helpful to the family members. It is important to communicate these recommendations in an articulate manner, whether verbally or in the written report.

Adolescent↗
PubMed2005

Di- and trivalent organogermanium reactive intermediates. Kinetics and mechanisms of some reactions of diphenylgermylene and tetraphenyldigermene in solution.

The reactivity of diphenylgermylene (Ph2Ge) with several classes of germylene scavengers has been studied in hexane solution at 23 degrees C by laser flash photolysis of 3,4-dimethyl-1,1-diphenyl-1-germacyclopent-3-ene (1a), a clean and highly efficient precursor to the germylene and its dimer, tetraphenyldigermene (2a). The reactions studied include M-H insertion reactions with Group 14 hydrides (M = Si, Ge, Sn), halogen atom abstractions from bromo- and chlorocarbons, Lewis acid-base complexation with 1 degrees, 2 degrees, and 3 degrees aliphatic amines, and reaction with an aliphatic alkene, alkyne and diene, and oxygen. Absolute rate constants for (irreversible) scavenging of the germylene could be obtained by direct measurement of the germylene decay kinetics for all but the least efficient scavengers (triethylsilane, oxygen, chloroform, and 1-bromopentane), for which estimates of the rate constants were obtained by Stern-Volmer analysis of the reduction in digermene yield as a function of scavenger concentration. Distinctly different kinetic behavior is observed for scavenging of Ph2Ge by isoprene, 4,4-dimethyl-1-pentene, and triethylamine; in these cases, the results suggest that reaction is rapid (k(Q) = 3-6 x 10(9) M(-1)s(-1)) but reversible (K(eq) = 2500 - ca. 20,000 M(-1)) over the range of scavenger concentrations studied. The reactions with the C-C unsaturated compounds proceed via the intermediacy of long-lived transient species absorbing at <290 nm, which are tentatively assigned to the corresponding three-membered germanocycles on the basis of their UV spectra and lifetimes. Upper limits for the absolute rate constants for reaction of tetraphenyldigermene (2a) toward many of these reagents are also reported.

Journal Article↗
PubMed2004

Organogermanium reactive intermediates. The direct detection and characterization of transient germylenes and digermenes in solution.

Diphenylgermylene (Ph2Ge) and its Ge=Ge doubly bonded dimer, tetraphenyldigermene (6a), have been characterized directly in solution for the first time by laser flash photolysis methods. The germylene is formed via (formal) cheletropic photocycloreversion of 3,4-dimethyl-1,1-diphenylgermacyclopent-3-ene (4a), which is shown to proceed in high chemical (>95%) and quantum yield (phi = 0.62) by steady-state trapping experiments with methanol, acetic acid, isoprene, and triethylsilane. Flash photolysis of 4a in dry deoxygenated hexane at 23 degrees C leads to the prompt formation of a transient assigned to Ph2Ge (lambda(max) = 500 nm; epsilon(max) = 1650 M(-1) cm(-1)), which decays with second-order kinetics (tau approximately 3 micros), with the concomitant growth of a second transient species that is assigned to digermene 6a (tau approximately 40 micros; lambda(max) = 440 nm). Analogous results are obtained from 1,1-dimesityl- and 1,1-dimethyl-3,4-dimethylgermacyclopent-3-ene (4b and 4c, respectively), which afford Mes2Ge (tau approximately 20 micros; lambda(max) = 560 nm) and Me2Ge (tau approximately 2 micros; lambda(max) = 480 nm), respectively, as well as the corresponding digermenes, tetramesityl- (6b; lambda(max) = 410 nm) and tetramethyldigermene (6c; lambda(max) = 370 nm). The results for the mesityl compound are compared to the analogous ones from laser flash photolysis of the known Mes2Ge/6b precursor, hexamesitylcyclotrigermane. The spectra of the three germylenes and two of the digermenes are in excellent agreement with calculated spectra, derived from time-dependent DFT calculations. Absolute rate constants for dimerization of Ph2Ge and Mes2Ge and for their reaction with n-butylamine and acetic acid in hexane at 23 degrees C are also reported.

Journal Article↗
PubMed2002

Complexities in ETS-domain transcription factor function and regulation: lessons from the TCF (ternary complex factor) subfamily. The Colworth Medal Lecture.

The ETS-domain transcription factor family can be divided into a series of subfamilies. Elk-1 represents the founding member of the ternary complex factor (TCF) subfamily. By focusing on the TCF subfamily, we can demonstrate the complexities that exist in the function and regulation of ETS-domain transcription factors. This article focuses on Elk-1 in detail and summarizes the functions of other TCFs. The key themes covered include the domain structure of the TCFs, the mechanisms of complex formation with serum response factor, regulation of TCFs by mitogen-activated protein kinase cascades, and transcriptional regulatory properties of the TCFs. Finally, the emerging role of the TCFs in vivo is discussed. A picture is developing indicating that, while these proteins exhibit significant sequence and functional conservation, key differences in their structure and regulation are being identified which may relate to unique functions of these proteins in vivo.

Amino Acid Sequence↗
PubMed2005

The role of calcium antagonists in stroke prevention.

Lowering elevated blood pressure significantly reduces the risk of stroke, but whether any specific type of antihypertensive medication is better for this purpose, independent of blood pressure-lowering effects, is controversial. Compared with placebo or no treatment, the point estimate for stroke reduction is the largest with calcium antagonists; all such studies used dihydropyridine compounds. When all clinical trials published through December 2004 are combined in meta-analysis, stroke is not significantly reduced when an initial dihydropyridine or nondihydropyridine calcium antagonist is compared with an initial diuretic or beta blocker, but when the two subclasses are combined, stroke reduction (9%) is significant. The risk of heart failure, however, is significantly increased (by 29%) with the initial calcium antagonist. These estimates may change as newer trials are reported.

Adrenergic beta-Antagonists↗
PubMed2006

Combining multistate capture-recapture data with tag recoveries to estimate demographic parameters.

Matrix population models that allow an animal to occupy more than one state over time are important tools for population and evolutionary ecologists. Definition of state can vary, including location for metapopulation models and breeding state for life history models. For populations whose members can be marked and subsequently reencountered, multistate mark-recapture models are available to estimate the survival and transition probabilities needed to construct population models. Multistate models have proved extremely useful in this context, but they often require a substantial amount of data and restrict estimation of transition probabilities to those areas or states subjected to formal sampling effort. At the same time, for many species, there are considerable tag recovery data provided by the public that could be modeled in order to increase precision and to extend inference to a greater number of areas or states. Here we present a statistical model for combining multistate capture-recapture data (e.g., from a breeding ground study) with multistate tag recovery data (e.g., from wintering grounds). We use this method to analyze data from a study of Canada Geese (Branta canadensis) in the Atlantic Flyway of North America. Our analysis produced marginal improvement in precision, due to relatively few recoveries, but we demonstrate how precision could be further improved with increases in the probability that a retrieved tag is reported.

Animal Migration↗
PubMed2003

Symptom patterns among Gulf War registry veterans.

OBJECTIVES: We identify symptom patterns among veterans who believe they suffer from Gulf War-related illnesses and characterize groups of individuals with similar patterns. METHODS: A mail survey was completed by 1161 veterans drawn from the Gulf War Health Registry. RESULTS: An exploratory factor analysis revealed 4 symptom factors. A K-means cluster analysis revealed 2 groups: (1) veterans reporting good health and few moderate/severe symptoms, and (2) veterans reporting fair/poor health and endorsing an average of 37 symptoms, 75% as moderate/severe. Those in Cluster 2 were more likely to report having 1 or more of 24 medical conditions. CONCLUSIONS: These findings are consistent with previous investigations of symptom patterns in Gulf War veterans. This multisymptom illness may be more fully characterized by the extent, breadth, and severity of symptoms reported.

Chronic Disease↗
PubMed2004

Normative data for the selective reminding test: a random digit dialing sample.

Healthy control participants (46 women, M age=44.3 yr., SD=7.6; 29 men) were recruited to undergo a comprehensive neuropsychological battery and serve as a comparison group in a study of cognitive functioning in patients with Lyme isease. Participants were selected using Mitofsky-Waksberg random digit dialing. The Buschke 12-word, six-trial Selective Reminding Test was administered as part of the neuropsychological battery and normative data are presented stratified by age and sex. Performance on alternate forms of this measure were examined. Mean education, intelligence quotient, and Wide Range Achievement Test-3 Reading scores are reported.

Achievement↗
PubMed1997

Genotype-phenotype correlation in two sets of monozygotic twins with Williams syndrome.

We report on two sets of monozygotic (MZ) twins with Williams syndrome (WS), following the 6 pairs already reported in the literature. We have confirmed monozygosity of both pairs of twins by DNA microsatellite analysis and the clinical diagnosis by fluorescence in situ hybridization using a WS-specific probe. Analysis of the concordance of different clinical signs between members of each pair of twins benefitted from a lengthy clinical follow-up, from 24 months to 7 years in one pair, and from the age of 15 years with reevaluation after 2 years in the other pair. Most clinical signs were concordant in the twins of each pair, with differences present at younger ages, mainly minor facial anomalies, being attenuated with time. Developmental delay was substantially concordant, but the degree differed slightly between twins in each pair. Inguinal hernia was present in a single twin in pair 1. Facial anomalies and other signs attributable to connective tissue abnormalities were also displayed by only one twin in both sets, suggesting that the WS genotype has only a predisposing role in the development of these signs.

Adolescent↗