Midazolam infusion in a pediatric intensive care unit.
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Child↗
Biomedical subjects
K Walsh
Publications and source records attributed to K Walsh.
Long-term administration of valproic acid in the treatment of affective symptoms in people with mental retardation.
We report the results of an open trial of valproic acid in the treatment of affective symptoms in people with mental retardation. The study population consisted of 209 people with mental retardation who were serially referred to a tertiary-care medical center for the evaluation of behavioral symptoms. Criteria for treatment included the presence of three of the four following symptoms: irritability, sleep disturbance, aggressive or self-injurious behavior, and behavioral cycling. Twenty-one patients met enrollment criteria and were studied prospectively for a 2-year period. Two patients were lost to follow-up. One patient experienced severe drug side effects. Eighteen patients completed the study. Fourteen patients (78%) responded favorably to treatment and were maintained on valproic acid for the 2 years of the study (p < 0.001). Medications prescribed at the time of enrollment were usually discontinued, including neuroleptic medication in 9 of 10 patients and in all patients (N = 3) who were receiving phenobarbital. A history of epilepsy or a suspicion of seizures was strongly associated with a favorable response to valproic acid (p < 0.005). The results of this study suggest that people with mental retardation and concurrent affective disorders can be recognized by a cluster of developmentally appropriate symptoms such as those listed above. In addition, affective symptoms can be successfully treated with valproate with reductions in neuroleptic and barbiturate anticonvulsant medication. Further study of the comparative benefit of valproate and carbamazepine in this population is warranted.
Adolescent↗
Molecular cloning of a homeobox transcription factor from adult aortic smooth muscle.
We report here the cloning of a cDNA encoding a homeobox transcription factor from vascular smooth muscle and describe its unique pattern of mRNA expression at different stages in development. The cDNA isolated is 1576 base pairs in length not including the poly(A) tail and contains an open reading frame coding for a predicted 372-amino acid homeobox protein. During early embryogenesis, expression was detected in the neural tube with a sharp expression boundary occurring at an anterior position, in the myelencephalon, in the third and fourth branchial arches, and in vessels leading from the heart. In adults, however, transcripts were only detected in aortic smooth muscle and lung but were undetectable in cardiac or skeletal muscle, visceral smooth muscle, and many other tissues including brain. In neonates, expression was detected in the outflow tracts of the heart as well as in the cardiomyocytes. The expression pattern of this gene suggests that, although it likely has multiple roles during development, in the adult, it may participate in the control of vascular smooth muscle differentiation and proliferation.
Aging↗
MyoD binds to the guanine tetrad nucleic acid structure.
A high affinity interaction between a protein and the guanine tetrad nucleic acid structure is described. Recombinant MyoD, a transcription factor that can initiate myogenesis, specifically bound to helical structures formed by stacks of guanine residues in square planar arrays. The N-7 methylation of a set of consecutive dG residues in a single-stranded probe of the creatine kinase enhancer interfered with the formation of this nucleic acid structure and prevented protein binding. Recombinant MyoD also bound to a guanine tetrad formed with a telomeric DNA probe, and it had a higher affinity for the four-stranded structure than for the double-stranded E-box-binding site. These data are the first report of a direct interaction between a protein and this nucleic acid conformation. The potential biological significance of this finding is discussed.
Animals↗
Primary structure and functional expression of the beta 1 subunit of the rat brain sodium channel.
Voltage-sensitive sodium channels are responsible for the initiation and propagation of the action potential and therefore are important for neuronal excitability. Complementary DNA clones encoding the beta 1 subunit of the rat brain sodium channel were isolated by a combination of polymerase chain reaction and library screening techniques. The deduced primary structure indicates that the beta 1 subunit is a 22,851-dalton protein that contains a single putative transmembrane domain and four potential extracellular N-linked glycosylation sites, consistent with biochemical data. Northern blot analysis reveals a 1,400-nucleotide messenger RNA in rat brain, heart, skeletal muscle, and spinal cord. Coexpression of beta 1 subunits with alpha subunits increases the size of the peak sodium current, accelerates its inactivation, and shifts the voltage dependence of inactivation to more negative membrane potentials. These results indicate that the beta 1 subunit is crucial in the assembly, expression, and functional modulation of the heterotrimeric complex of the rat brain sodium channel.
Amino Acid Sequence↗
Patency of Blalock-Taussig shunt assessed by Doppler colour flow imaging.
A neonate with pulmonary atresia and intact ventricular septum underwent a modified right sided Blalock-Taussig shunt. Following the procedure, the patient developed a right sided haemothorax, which required drainage. A murmur was not audible subsequently. Echocardiography with colour flow imaging enabled identification of patency of the right sided shunt. It was then possible to discontinue intravenous prostaglandin medication.
Blood Vessel Prosthesis↗
Absent left-sided atrioventricular connexion, with right atrium connected to left ventricle: prospective diagnosis in infancy, and outcome.
Prospective echocardiographic diagnosis of absence of the left atrioventricular connexion, with the right atrium connected to a morphologic left ventricle through a bileaflet morphologically mitral valve, was made in six infants. The rudimentary right ventricle was left-sided in all patients, and separated from the left atrium by sulcus tissue. The ventriculoarterial connexions were discordant. Associated defects included subpulmonary stenosis (2 patients), pulmonary atresia (1 patient), and a patent duct (4 patients). All patients developed early left atrial hypertension due to a restrictive interatrial septum, and required transcatheter septostomy (5 patients), or surgical septectomy (3 patients). One patient who had a severely restrictive ventricular septal defect died following cardiac catheterization. In three others the ventricular septal defect has become progressively restrictive on serial catheterization. Successful intermediate term palliation has been performed in two patients using a bidirectional Glenn anastomosis, together with enlargement of the ventricular septal defect and a Damus-Kay-Stansel procedure in one. It is possible to distinguish this malformation from "mitral atresia" using cross-sectional echocardiography. The long-term outlook is influenced by early relief of left atrial hypertension. Balloon atrial septostomy alone is usually inadequate, and either blade septostomy or surgical septectomy are required. Serial cardiac catheterization is mandatory for planning definitive palliation.
Cardiac Catheterization↗
Diagnosis of total anomalous pulmonary venous drainage by Doppler color flow imaging.
Over a 2.5-year period, 16 consecutive infants were prospectively diagnosed as having total anomalous pulmonary venous drainage. The sites of drainage were cardiac (to the coronary sinus) in four patients, supracardiac in nine, infracardiac in two and mixed in one patient. In every case, two-dimensional echocardiography with color flow imaging enabled complete and correct diagnosis of the sites of drainage and the presence or absence of pulmonary venous obstruction. The echocardiographic findings were verified at surgery or autopsy in all. Color flow imaging rapidly provided information about the direction and mean velocity of flow through abnormal vascular structures in any two-dimensional echocardiographic plane. It facilitated the acquisition of quantitative velocity information with standard Doppler ultrasound techniques by identifying areas of high velocity or turbulent flow and was invaluable in the assessment of anomalous pulmonary venous drainage occurring either as an isolated anomaly or in conjunction with complex intracardiac lesions.
Blood Flow Velocity↗
Profiles, correlates, and trajectories of intelligence in Prader-Willi syndrome.
Three aspects of intellectual functioning in persons with Prader-Willi syndrome were examined in two, related studies. In study 1, 21 subjects were evaluated with a psychometric instrument that assesses neuropsychological styles of cognitive processing, the Kaufman Assessment Battery for Children. Prader-Willi subjects showed deficits in sequential processing, and strengths in academic achievement tasks such as reading and vocabulary. In contrast to previous reports on the syndrome, no relationship was found between weight and degree of intellectual impairment. Study 2 included a cross-sectional examination of the trajectory of IQ in 21 subjects aged 13 to 46 years, as well as a longitudinal analysis of 31 subjects aged 5 to 30 years who were tested twice with the same IQ test. No evidence of the previously described decline in IQ over time was noted in either the cross-sectional or longitudinal analyses. The implications of these findings for interventions are discussed.
Achievement↗
Adaptive and maladaptive behavior in Prader-Willi syndrome.
The development and profiles of adaptive and maladaptive behavior of 21 adolescents and adults with Prader-Willi syndrome were cross-sectionally examined with the Vineland Adaptive Behavior Scales and Achenbach's Child Behavior Checklist (CBCL). Adaptive strengths emerged for the group as a whole in daily living skills, and this strength became more pronounced with increasing age. A relative weakness was found in socialization, most notably in coping skills. CBCL findings indicated that externalizing behaviors were particularly heightened in adolescence and that many behaviors previously described as either emerging or worsening in adolescence also persist into the adult years (e.g., temper tantrums, arguing, irritability, stubbornness, lying, skin picking, obsessions, defiance). Certain elevated CBCL behaviors were unique to young versus old age groups, and aging in this syndrome may be associated with heightened confusion, withdrawal, and fatigue. The need to study adaptive and maladaptive features in a wider age range of subjects with Prader-Willi syndrome was emphasized.
Activities of Daily Living↗
Nucleic acid specificity of a vertebrate telomere-binding protein: evidence for G-G base pair recognition at the core-binding site.
A factor from avian cells formed complexes with telomeric sequences and other single-stranded probes that contained tracts of guanine residues. Nucleoprotein complexes with telomere probes required two or more of the telomeric repeats that were incapable of Watson-Crick base-pairing. Methylation interference and protection experiments identified guanine N7 residues that were critical for the formation of the nucleoprotein complex and for the formation of a higher-order structure that occurred in the absence of the protein. Substitutions of deoxyinosine (dI) for deoxyguanosine (dG) demonstrated that the exocyclic N2 amino groups in the internal telomeric repeat, but not the terminal repeat, were required for the formation of the chemically protected structure and for protein binding. On the basis of these data we propose that the factor specifically recognizes a hairpin DNA structure that is stabilized by intramolecular G-G base-pairing between the telomere repeats. The positions of the critical guanine N2 and N7 groups indicate a G-G base-pairing configuration, where guanines function as hydrogen bond donors at the internal telomeric repeat and hydrogen bond acceptors at the terminal telomeric repeat.
Animals↗
Functional antagonism between YY1 and the serum response factor.
The rapid, transient induction of the c-fos proto-oncogene by serum growth factors is mediated by the serum response element (SRE). The SRE shares homology with the muscle regulatory element (MRE) of the skeletal alpha-actin promoter. It is not known how these elements respond to proliferative and cell-type-specific signals, but the response appears to involve the binding of the serum response factor (SRF) and other proteins. Here, we report that YY1, a multifunctional transcription factor, binds to SRE and MRE sequences in vitro. The methylation interference footprint of YY1 overlaps with that of the SRF, and YY1 competes with the SRF for binding to these DNA elements. Overexpression of YY1 repressed serum-inducible and basal expression from the c-fos promoter and repressed basal expression from the skeletal alpha-actin promoter. YY1 also repressed expression from the individual SRE and MRE sequences upstream from a TATA element. Unlike that of YY1, SRF overexpression alone did not influence the transcriptional activity of the target sequence, but SRF overexpression could reverse YY1-mediated trans repression. These data suggest that YY1 and the SRF have antagonistic functions in vivo.
Animals↗
Giant right atrial diverticulum: an unusual cause of Wolff-Parkinson-White syndrome.
A case of a giant right atrial diverticulum associated with neonatal supraventricular tachycardia is reported. The electrocardiogram in sinus rhythm showed pre-excitation that may have been caused by the right atrial diverticulum adhering to the right ventricle.
Adult↗
Low back pain in eight areas of Britain.
STUDY OBJECTIVE: The aim was to assess the geographical variation in low back pain and associated disability in Britain. DESIGN: This was a cross sectional survey with information collected by postal questionnaire. SETTING: General practices in seven British towns and one rural district. SUBJECTS: 1172 men and 1495 women aged 20-59 years were selected from the age-sex registers of 136 general practitioners in the study areas. MAIN RESULTS: The overall lifetime and one year period prevalences of low back pain were 58.3% and 36.1%. Rates in men and women were similar. Symptoms were more common in men with manual occupations than in those with non-manual jobs, but in women there was no clear trend in relation to social class. Geographical differences in prevalence were small, but the threshold for consulting general practitioners about symptoms varied markedly from place to place. After allowance for age, sex, social class, and severity of symptoms, subjects in the northern towns of Arbroath and Peterlee who had suffered from low back pain in the past year were three to four times as likely to have consulted their doctor about the problem as those living in the southern towns of St Austell and Dorking. Consultation rates in the Midlands were intermediate. CONCLUSIONS: Geographical variation in rates of general practice consultation for low back pain in Britain is due largely to differences in patient behaviour once symptoms have developed. The distribution of important causes of low back back pain across the country is probably fairly uniform.
Adult↗
Risk of low back pain in people admitted to hospital for traffic accidents and falls.
STUDY OBJECTIVE: The aim was to assess the risk of back symptoms in people admitted to hospital because of traffic accidents and falls. DESIGN: The study was a cross sectional survey with information collected by postal questionnaire. Main outcome measures were associations between hospital admission for a traffic accident or fall and reported first onset of back symptoms at the same age and at later ages. SETTING: General practices in seven towns and one rural district. SUBJECTS: 1172 men and 1495 women aged 20-59 years were selected from the age-sex registers of 136 general practitioners in the study areas. MAIN RESULTS: Low back pain was reported by 1556 subjects and hospital admission for a traffic accident or fall by 362. The incidence of low back pain was unusually high during the year of age at which subjects were first admitted to hospital for trauma (RR = 5.5, 95% CI 3.8-7.8). The risk of first developing symptoms in subsequent years was lower, but still significantly increased (RR = 1.3, 95% CI 1.1-1.6). Low back pain which started at the age of an accident tended to last longer than that occurring in other circumstances, and was more often ascribed to injury (56% of cases). However, this proportion was smaller than the calculated attributable proportion for traffic accidents and falls (82%). CONCLUSIONS: The data suggest that a person under age 60 years who is admitted to hospital for a traffic accident or fall has a 7% chance of developing low back pain as result of the injury. However, the link between the injury and subsequent symptoms is often not obvious to the patient.
Accidental Falls↗
Health care fraud: security management's role.
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Computer Systems↗
Professional challenges in the coming decade.
Last month, as part of our observance of the 15th anniversary of Physician Assistant journal, members of our Editorial Board offered their views on turning points in the history of the PA profession. In this issue, we jump from the past to the future, with perspectives on the main crises or changes facing the profession in the coming decade. You may agree or disagree with these viewpoints. Diversity of opinion keeps the PA profession dynamic and strong--which is exactly why Physician Assistant journal provides an open forum for letters and guest editorials.
Forecasting↗
A prospective cytogenetic study of 36 cases of DiGeorge syndrome.
Cytogenetic analysis was carried out in a prospective series of 36 children with DiGeorge syndrome. High-resolution banding (> 850 bands/haploid set) was achieved in 30 cases. Monosomy 22q11.21-->q11.23 was found in 9 of these 30 cases. In each of these cases monosomy 22q11.21-->q11.23 resulted from an interstitial deletion and not from a translocation. No other chromosome abnormalities were seen.
Child↗