bluuebunny/crossref_metadata_2025_split huggingface dataset:
Validity of the CAGE questionnaire in hospital
Authors: Malet Laurent , Schwan Raymund , Boussiron Didier , Aublet-Cuvelier Bruno , Llorca Pierre-Michel 
Published: September 2004

AbstractObjectiveThe CAGE questionnaire is considered a useful screening and case-finding tool for alcohol use disorders in clinical populations. Our objectives were to validate the French version of the CAGE against DSM-IV criteria and to assess performance of each item of the scale.MethodData were extracted from a hospital morbidity study conducted in central France. It concerned 5452 patients—48.5% men—in short and medium-stay units. Patients answered the CAGE questionnaire as a past-year assessment. The alcohol use disorders were diagnosed by the physicians using DSM-IV alcohol abuse or dependency criteria.ResultsThe CAGE questionnaire for a cut-off of 2 had a sensitivity of 77% and a specificity of 94%. The CAGE test was more sensitive for patients diagnosed as alcohol-dependent than for alcohol abusers (61% vs. 84%) with the same specificity (94%). These values are close to those for the English-language CAGE. The first three items (CAG) were very similar, with sensitivity 70% and specificity 94%. The eye-opening question (E) differentiated sharply between abuse and dependency, with sensitivities of 18% and 46%, respectively. A questionnaire comprising only the CAG questions of the CAGE had properties similar to the full questionnaire.ConclusionCAGE is a good screening tool for alcohol abuse or alcohol dependency. Given the frequent—and insufficiently diagnosed—alcohol problems among inpatients, CAGE is indicated as a first-line tool for screening for the most severe alcohol use disorders in hospital. It should ideally be used systematically. A positive reply to any of the first three items should alert the clinician and prompt further investigation.
Involuntarily and voluntarily admitted patients’ experiences of psychiatric admission and treatment—a comparison before and after changed legislation in Sweden
Authors: Wallsten Tuula , Kjellin Lars 
Published: September 2004

AbstractObjective– The Swedish Compulsory Mental Care Act (LPT) of 1992 emphasises the participation of patients and relatives in the treatment of the patients. The purpose of this study was to compare patients' experiences under the LPT with patients' experiences under the previous law.Method.– Samples of 44 committed patients and 40 voluntarily admitted patients in 1991 and 49 committed and 49 voluntarily admitted patients in 1997/98 were interviewed at admission and at discharge, or after 3 weeks of care.Results.– There were similar proportions of committed and voluntarily admitted patients, respectively, in 1991 and 1997/98 who reported participation in treatment planning and participation of relatives and who reported deprivation of liberty, but more committed patients in 1997/98 reported coercive measures.Conclusion.– There were few differences of the patient's experiences between the study occasions. The fundamental aims of the legislation have not been fulfilled.
Toward an integrative approach to designing service experiences
Authors: Stuart F. Ian , Tax Stephen 
Published: September 2004

AbstractA critical element in designing a new service and ultimately achieving customer delight and loyalty, particularly for competitive services where the customer–service provider interface is high, deals with the delivery of memorable personal experience. Since many such services rely on attributes of theatre‐like performance, this research examines the process by which theatre plays are chosen, designed and developed with the objective of better understanding how performance excellence is realized. Theatre plays were chosen as a basis for study because of their long history of mastering the critical elements of performance staging and the reputation for opening on time at near peak performance levels. Using a case‐based, qualitative research approach, the theatre play process is described, implications are extracted and a corresponding management process model is presented. Research and management implications for new service design and service delivery excellence are suggested.
Successful long‐term treatment of hepatic carnitine palmitoyltransferase I deficiency and a novel mutation
Authors: Stoler J. M. , Sabry M. A. , Hanley C. , Hoppel C. L. , Shih V. E. 
Published: September 2004

AbstractSummary: Individuals with carnitine palmitoyltransferase I (CPT‐I) deficiency cannot metabolize long‐chain fatty acids and can develop life‐threatening hypoglycaemia. We present a boy with CPT‐I deficiency maintained on a very low‐fat diet with nighttime uncooked cornstarch feedings for 5 1/2 years with good success. He has had normal growth and no episodes of hypoglycaemia or adverse side‐effects. We found that he was homozygous for a previously undescribed mutation, T314I, in the CPT1A protein.
Congenital disorder of glycosylation (CDG) type Ie. A new patient
Authors: García‐Silva M. T. , Matthijs G. , Schollen E. , Cabrera J. C. , del Pozo J. Sanchez , Herreros M. Martí , Simón R. , Maties M. , Hernández E. Martín , Hennet T. , Briones P. 
Published: September 2004

AbstractSummary: CDG Ie is caused by a deficiency of dolichol‐phosphate‐mannose synthase 1 (DPM1), an enzyme involved in N‐glycan assembly in the endoplasmic reticulum. Three proteins are known to be part of the synthase complex: DPM1, DPM2 and DPM3. Only mutations in DPM1, the catalytic subunit, have been described in three families. One was homozygous for the c274C>;G (R92G) mutation in DPM1 and two others were compound heterozygous for R92G and a c628delC deletion or a c331–343del13, respectively. Clinical features were a severe infantile encephalopathy, early intractable seizures, acquired microcephaly, and some dysmorphic features. We report a patient with milder symptoms: microcephaly, dysmorphic features, developmental delay, optic atrophy, and cerebellar dysfunction without cerebellar atrophy. The patient is homozygous for a new mutation in exon 9 of the DPM1 gene (c742T>;C (S248P)). Our findings extend the spectrum of CDG Ie.
Neuropsychological outcome of subjects participating in the PKU Adult Collaborative Study: A preliminary review
Authors: Brumm V. L. , Azen C. , Moats R. A. , Stern A. M. , Broomand C. , Nelson M. D. , Koch R. 
Published: September 2004

AbstractSummary: Adult subjects with classical phenylketonuria (PKU) who were diagnosed and treated neonatally participated in this long‐term follow‐up study. Twenty‐four subjects received neuropsychological (NP) assessment and a subset received magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) to identify: (1) pattern of cognitive dysfunction; (2) effect of high blood phenylalanine (Phe) level at time of cognitive testing; and (3) treatment variables that may be associated with cognitive difficulties in adulthood. All subjects had average IQ except one subject in the borderline range. Diet was initiated by the 15th day of life. All subjects except one were on diet until age 6 years (mean years of treatment = 15). Blood Phe levels at cognitive testing ranged from 157 to 1713 µmol/L (mean = 1038); 11 subjects had levels >1000 µmol/L and 13 subjects had levels >;1000 µmol/L. Results suggest that adults with early‐treated PKU demonstrate specific cognitive deficits, a number of which are associated with the frontal and temporal area of the brain. Deficits were noted in several domains including executive functioning, attention, verbal memory, expressive naming and verbal fluency. Self‐report measures of depression and anxiety were generally in the normal/mild range. The group with a Phe level >;1000 µmol/L scored lower than the group with Phe level <1000 µmol/L on measures of focused attention, verbal fluency, reaction time, verbal recognition memory, visual memory and naming. Tests of cognitive functioning were often correlated with measures of treatment during childhood rather than with Phe level at the time of cognitive testing. Subjects with abnormal MRI scored significantly lower on two cognitive tests (Trails A and CVLT Recognition Memory). We found no significant correlation between current brain Phe level obtained through MRS (n = 10) and neuropsychological functioning. Future longitudinal investigation with a larger sample size will assist in clarifying the aetiology of neuropsychological deficits and association with treatment history.
A simple and rapid enzymatic assay for the branched‐chain α‐ketoacid dehydrogenase complex using high‐performance liquid chromatography
Authors: Tajima G. , Yofune H. , Bahagia Febriani A. D. , Nishimura Y. , Ono H. , Sakura N. 
Published: September 2004

AbstractSummary: Maple syrup urine disease (MSUD) is caused by a congenital defect of the branched‐chain α‐ketoacid dehydrogenase complex (BCKADC), and is one of the target disorders in newborn screening. However, it is not always easy to confirm the diagnosis; conventional methods of enzyme assay require cell culture, isolation of mitochondria, or radioisotope‐labelled reagents, and disease‐causing mutations can exist in any of the genes encoding the three enzyme subunits. To realize a practical test for diagnostic confirmation, we developed a simple and rapid enzymatic assay for BCKADC. In this procedure, the production of isovaleryl‐CoA from 2‐ketoisocaproic acid was measured using high‐performance liquid chromatography. Detection of the BCKADC product was significantly reproducible depending on concentration of the substrates. We applied the assay to two patients with MSUD and demonstrated pathologically low levels of residual activity in both subjects. These results indicate that our method is a practical and sensitive assay for BCKADC, and that it can be a useful adjunct in newborn screening for MSUD.
Noncirrhotic portal hypertension in association with juvenile nephropathic cystinosis: Case presentation and review of the literature
Authors: DiDomenico P. , Berry G. , Bass D. , Fridge J. , Sarwal M. 
Published: September 2004

AbstractSummary: We report a case of portal hypertension and oesophageal varices arising in an 18‐year‐old female renal transplant recipient with juvenile nephropathic cystinosis diagnosed at 6 years of age. The patient had a history of poor compliance with her prescribed cysteamine therapy. Routine examination revealed normal liver function without hepatomegaly but asymptomatic splenomegaly. An abdominal ultrasound suggested mild oesophageal varices, confirmed later on endoscopy. A liver biopsy revealed an abundance of cystine crystals within the hepatic Kupffer cells, with preserved hepatic architecture. Although the pathophysiology of this rare complication is unclear, in the absence of other aetiologies the likely cause is the patient's poorly controlled cystinosis. As cystinotic patients live longer with improved renal transplant management and cysteamine therapy, it is of interest to characterize the long‐term course of the illness after renal transplantation. An understanding of the pathophysiology of hepatic dysfunction will be required to manage this potential late complication of the disease.
Decomposition Analyses Applied to a Complex Ultradian Biorhythm: The Oscillating NADH Oxidase Activity of Plasma Membranes Having a Potential Time-Keeping (Clock) Function
Authors: Foster Ken , Anwar Nasim , Pogue Rhea , Morré Dorothy M. , Keenan T. W. , Morré D. James 
Published: September 2004

Seasonal decomposition analyses were applied to the statistical evaluation of an oscillating activity for a plasma membrane NADH oxidase activity with a temperature compensated period of 24 min. The decomposition fits were used to validate the cyclic oscillatory pattern. Three measured values, average percentage error (MAPE), a measure of the periodic oscillation, mean average deviation (MAD), a measure of the absolute average deviations from the fitted values, and mean standard deviation (MSD), the measure of standard deviation from the fitted values plus R-squared and the Henriksson-Merton p value were used to evaluate accuracy. Decomposition was carried out by fitting a trend line to the data, then detrending the data if necessary, by subtracting the trend component. The data, with or without detrending, were then smoothed by subtracting a centered moving average of length equal to the period length determined by Fourier analysis. Finally, the time series were decomposed into cyclic and error components. The findings not only validate the periodic nature of the major oscillations but suggest, as well, that the minor intervening fluctuations also recur within each period with a reproducible pattern of recurrence.
Auditory Brainstem Response versus Magnetic Resonance Imaging for the Evaluation of Asymmetric Sensorineural Hearing Loss
Authors: Cueva Roberto A. 
Published: September 2004

AbstractObjectives/Hypothesis: Auditory brainstem response (ABR) testing and magnetic resonance imaging (MRI) are compared for the evaluation of patients with asymmetric sensorineural hearing loss (SNHL). MRI with gadolinium administration is the current gold standard for identifying retrocochlear lesions causing asymmetric SNHL. The study seeks to determine the sensitivity and specificity of ABR in screening for possible retrocochlear pathology. Most important among SNHL etiologies are neoplastic lesions such as vestibular schwannomas, cerebellopontine angle (CPA) tumors, as well as multiple sclerosis, stroke, or other rare nonneoplastic causes. The study results will allow the author to recommend a screening algorithm for patients with asymmetric SNHL.Study Design: The study is a multi‐institutional, institutional review board approved, prospective, nonrandomized comparison of ABR and MRI for the evaluation of patients with asymmetric SNHL.Methods: Three hundred twelve patients (between the ages of 18 and 87) with asymmetric SNHL completed the study. Asymmetric SNHL was defined as 15 dB or greater asymmetry in two or more frequencies or 15% or more asymmetry in speech discrimination scores (SDS). These patients prospectively underwent both ABR and MRI. The ABR and MRI were interpreted independently in a blinded fashion. In addition to the ABR and MRI results, a variety of clinical and demographic data were collected.Results: Thirty‐one (9.94%) patients of the study population of 312 were found on MRI to have lesions causing their SNHL. Of the 31 patients with causative lesions on MRI there were 24 vestibular schwannomas, 2 glomus jugulare tumors, 2 ectatic basilar arteries with brainstem compression, 1 petrous apex cholesterol granuloma, 1 case of possible demyelinating disease, and 1 parietal lobe mass. Twenty‐two of the 31 patients had abnormal ABRs, whereas 9 patients (7 with small vestibular schwannomas) had normal ABRs. This gives an overall false‐negative rate for ABR of 29%. The false‐positive rate was found to be 76.84%. Sensitivity of ABR as a screening test was 71%, and specificity was 74%.Conclusions: Ten percent of patients with asymmetric SNHL (by this study's criteria) are likely to have causative lesions found on MRI. Although the recently reported annual incidence of vestibular schwannoma in the general population is 0.00124%, for patients with asymmetric SNHL in this study, the incidence was 7.7% (nearly 4 orders of magnitude higher). ABR has been demonstrated to have low sensitivity and specificity in the evaluation of these patients and cannot be relied on as a screening test for patients with asymmetric SNHL. Keeping the use of MRI conditional on the results of ABR will annually result in missed or delayed diagnosis of causative lesions in 29 patients per 1,000 screened. The author recommends abandoning ABR as a screening test for asymmetric SNHL and adoption of a focused MRI protocol as the screening test of choice (within certain guidelines).
Factors Predicting Patient Perception of Dysphonia Caused by Benign Vocal Fold Lesions
Authors: Behrman Alison , Sulica Lucian , He Tina 
Published: September 2004

AbstractObjectives/Hypothesis: To assess factors that may be predictive of patient perception of dysphonia severity, as quantified by the Voice Handicap Index (VHI) score. We hypothesize that 1) level of vocal demand; 2) auditory‐perceptual evaluation of dysphonia severity; and 3) vocal function, as defined by phonatory glottal closure and mucosal wave vibration, are the most significant predictors of VHI score.Study Design: Retrospective review of 100 patients with benign vocal fold lesions.Methods: Variables assessed for predictive value to VHI score are level of vocal demands, auditory‐perceptual evaluation of dysphonia severity, integrity of mucosal wave vibration and phonatory glottal closure, lesion type, duration of current complaint, smoking, age, and sex. Harmonic to noise ratio was assessed in a subset of 50 patients.Results: Patients with routine voice use had significantly lower VHI scores than those with more intensive (nonsinging/acting) vocal demands. Patients who quit smoking had greater VHI scores than those who currently smoke or never started. Patients with long‐standing dysphonia tended to have lower VHI scores than those with shorter duration vocal complaints. Auditory‐perceptual assessment of dysphonia severity and harmonic to noise ratio were weak predictors of VHI score. Age, sex, lesion type, phonatory glottal closure, and mucosal wave vibration were not significant predictors of VHI score.Conclusions: Patient perception of dysphonia severity is independent of many factors commonly assessed during the evaluation of voice disorders. It appears to be an important independent element in the assessment of the effect of a benign vocal fold lesion and critical to therapeutic decision‐making.
Diagnosis of Pneumonia With an Electronic Nose: Correlation of Vapor Signature With Chest Computed Tomography Scan Findings
Authors: Hockstein Neil G. , Thaler Erica R. , Torigian Drew , Miller Wallace T. , Deffenderfer Olivia , Hanson C William 
Published: September 2004

AbstractObjectives/Hypothesis: The electronic nose is a sensor of volatile molecules that is useful in the analysis of expired gases. The device is well suited to testing the breath of patients receiving mechanical ventilation and is a potential diagnostic adjunct that can aid in the detection of patients with ventilator‐associated pneumonia.Study Design: A prospective study.Methods: We performed a prospective study of patients receiving mechanical ventilation in a surgical intensive care unit who underwent chest computed tomography (CT) scanning. A single attending radiologist reviewed the chest CT scans, and imaging features were recorded on a standardized form. Within 48 hours of chest CT scan, five sets of exhaled gas were sampled from the expiratory limb of the ventilator circuit. The gases were assayed with a commercially available electronic nose. Both linear and nonlinear analyses were performed to identify correlations between imaging features and the assayed gas signatures.Results: Twenty‐five patients were identified, 13 of whom were diagnosed with pneumonia by CT scan. Support vector machine analysis was performed in two separate analyses. In the first analysis, in which a training set was identical to a prediction set, the accuracy of prediction results was greater than 91.6%. In the second analysis, in which the training set and the prediction set were different, the accuracy of prediction results was at least 80%, with higher accuracy depending on the specific parameters and models being used.Conclusion: The electronic nose is a new technology that continues to show promise as a potential diagnostic adjunct in the diagnosis of pneumonia and other infectious diseases.
Temporal Bone Fractures: Traditional Classification and Clinical Relevance
Authors: Ishman Stacey L. , Friedland David R. 
Published: September 2004

AbstractObjectives/Hypothesis: The objectives were to evaluate the clinical relevance of traditional temporal bone radiographic descriptors and to investigate the efficacy of an alternative fracture classification scheme.Study Design: Retrospective consecutive case series.Methods: Charts and computed tomography scans representing 155 temporal bone fractures at a level I trauma center were reviewed. Fracture types were correlated with clinical presentation and outcomes.Results: The traditional classification system (i.e., longitudinal, transverse, or mixed) correlated poorly with clinical findings such as facial nerve weakness and cerebrospinal fluid leakage. It also had limited utility in predicting conductive hearing loss and sensorineural hearing. An alternative schema distinguishing petrous from nonpetrous involvement demonstrated better correlation with these measures. Cerebrospinal fluid leak was 1.1 times more common in transverse than in longitudinal fractures but was 9.8 times more common in petrous than in nonpetrous fractures. Similarly, facial nerve injury more strongly correlated with fractures through the petrous temporal bone than did the other fracture types. Sensorineural hearing loss did not correlate with the transverse fracture classification but was significantly more prevalent in petrous fractures. Likewise, conductive hearing loss did not correlate with longitudinal fractures but was four times more common in the “middle ear” subcategory of nonpetrous fractures.Conclusion: Traditional temporal bone fracture descriptions correlate poorly with clinical findings. However, simply distinguishing petrous from nonpetrous involvement demonstrates significant correlation with the occurrence of serious sequelae of temporal bone fractures. Subcategories of mastoid and middle ear involvement further refine this classification schema to correlate with minor complications. This simple, radiographically based scheme better focuses clinical resources and attention toward more likely sequelae.
Central Effects of Residual Hearing: Implications for Choice of Ear for Cochlear Implantation
Authors: Francis Howard W. , Yeagle Jennifer D. , Brightwell Toni , Venick Holly 
Published: September 2004

AbstractObjectives/Hypothesis: The study tested the hypothesis that among patients with similar levels of residual hearing in the nonimplanted ear, speech perception outcome is the same whether or not the implanted ear has profound or severe levels of hearing loss.Study Design: Retrospective.Methods: Levels of hearing loss in postlingually deafened adults who had cochlear implantation at Johns Hopkins University (Baltimore, MD) between 1991 and 2002 were classified according to pure‐tone averages as bilateral severe (n = 20), severe‐profound (severe in one ear and profound in the other) (n = 23), and bilateral profound (n = 43). There was no significant difference in the age at onset and duration of deafness among the three patient groups. Individuals in the bilateral severe and severe‐profound groups had comparable levels of severe hearing loss in their nonimplanted ears, whereas those in severe‐profound and bilateral profound groups had comparable levels of profound hearing loss in their implanted ears. Speech perception performance was evaluated using words from the Consonant Nucleus Consonant word list, Hearing in Noise Test sentences in quiet, and Central Institute for the Deaf sentences through recorded presentation at 70 dB sound pressure level (SPL).Results: Despite the profound hearing loss of the implanted ear in the asymmetrical group, there was no significant difference in mean speech perception scores compared with the bilateral severe group within the first year after implant surgery. By comparison, the bilateral profound group had lower speech perception results compared with patients with residual hearing in one or both ears.Conclusion: The study results suggest that implantation of the profoundly deafened ear does not diminish the functional advantage conferred by residual hearing in a patient with asymmetrical hearing loss. Therefore, the central auditory pathway may be the site at which persistent auditory function has its most beneficial effects.