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Biomedical subjects

J Rosenfeld

Publications and source records attributed to J Rosenfeld.

At least 19 recordsLinked to original sources

[Auditory processing disorder (APD): first normative data for the standardised diagnosis in school-age children].

BACKGROUND: Disorders in auditory processing have gained importance over the past 10 years. A first consensus statement defined this disorder and described several of its features. At the present stage of research, tests are missing to discriminate between normal and disturbed auditory processing development. MATERIAL AND METHODS: A total of 142 children from the second and third grades (105 control group, 37 patients) were examined for audiological, auditory and cognitive development, as well as speech and writing abilities. RESULTS: For the first time, normative data were defined for children from 8-9 years of age, and characteristic aspects of auditory processing disorder (APD) could be quantified. CONCLUSION: This study supplies a test-battery with important elements for diagnosing APD under defined conditions.

Auditory Perceptual Disorders↗

Development of an impregnated reagent and automation of solid-phase analytical derivatization for carbonyls: proof of principle.

This study undertakes reduction of scale and automation of a solid-phase analytical derivatization of carbonyls with 2,4-dinitrophenylhyrazine on a styrene-divinylbenzene resin (XAD-2). Three processes are tested. In the batch process, an aqueous phase consisting of 50 microL of sample and 150 microL of reagent solution is contacted with 6 mg XAD-2 by shaking. An impregnated reagent consisting of 2,4-dinitrophenylhydrazine hydrochloride (DNPH) deposited on XAD-2 enables two additional processes. In-vial derivatization with an impregnated reagent requires shaking 50 microL of sample with 6 mg of the impregnated reagent and reduced the reaction time from 10 to 5 min. The third process involves packing impregnated reagent a mini-column and flowing 50 microL of sample through under positive pressure supplied by a Harvard Pump. This reduces sample preparation time to 1 min. Studies are conducted with worst-case model analytes: butanone, 2-pentanone, and malonyldialdehyde. The carbonyl of the two ketones is hindered, and, thus, these two compounds react very slowly with DNPH in aqueous solution. Malonyldialdehyde is highly water soluble, and it does not react in aqueous phase but also would not sorb onto XAD-2 for reaction. Nevertheless, derivatization/extraction of all model compounds any of the three procedures result in reproducible and high yields.

Automation, Laboratory↗

Health related quality of life and psychological outcome in patients treated for craniopharyngioma in childhood.

UNLABELLED: Patients with craniopharyngioma are at risk for many adverse effects related to the tumour's invasive behaviour and its proximity to many vital structures. Profound psychosocial problems, memory impairment, pituitary and hypothalamic dysfunction in addition to the physical handicap of visual loss are frequently recognized sequelae of craniopharyngioma treatment. OBJECTIVES: To examine health related quality of life (QoL) and psychological outcomes of patients treated for craniopharyngioma at the Royal Children's Hospital, Melbourne, between January 1980 and September 2003. PATIENTS: Seven (17.4%) of 46 (26 male) had died. Thirty-nine remained, of whom 30 were contactable. Eighteen of 30 (8 male), mean age 21.2 +/- 6.7 years, agreed to evaluation, of whom 16/18 (88.9%) had three or more pituitary hormone deficiencies, 11/18 had visual impairment and 9/18 obesity. MEASUREMENTS: The Adult GH-Deficient Assessment (AGHDA) and Psychological General Well-Being (PGWB) questionnaires were employed to assess quality of life in patients and age- and sex-matched healthy controls. Additional psychological assessment, including intellectual and academic skills, emotional function, and adaptive behaviour, had been undertaken in 12 patients at a previous time. RESULTS: High levels of physical morbidity and psychological disability were described. The General Health score of patients was significantly worse than for controls on PGWB (p = 0.025), anxiety was higher in those who had surgery alone (p = 0.008) and subjective QoL associated with GHD using AGHDA was lower (p = 0.006). Few craniopharyngioma survivors (18/30) were available for evaluation, demonstrating difficulties in attempts to assess this complex group. The discrepancy between results of objective and subjective measures of QoL is discussed in terms of adaptation to illness, disabilities and changed perception of life fulfilment. CONCLUSIONS: Craniopharyngioma and its treatment result in significant, complex medical, social, psychological and emotional difficulties. The degree of global disability is not reflected in subjective QoL reports for this group, highlighting the need for careful selection of assessment instruments.

Activities of Daily Living↗

Attentional and processing skills following traumatic brain injury in early childhood.

PRIMARY OBJECTIVE: Attentional deficits are common following TBI in adults. This study examined whether these skills are also vulnerable following early childhood injury, when such skills are rapidly developing. RESEARCH DESIGN: This study investigated attention 30 months post-TBI in pre-schoolers (n=56) and age-matched controls (n=26). TBI children were divided into mild, moderate and severe injury groups. METHODS AND PROCEDURE: Pre-injury data were collected at time of injury. Assessment of attention included the Continuous Performance Test, Wechsler Intelligence Scale and Personality Inventory for Children. MAIN OUTCOMES AND RESULTS: Severe TBI was associated with reduced accuracy and slowed processing, particularly on complex motor responses. Sustained attention was more intact. Parental reports indicate internalized behavioural problems and somatic complaints. CONCLUSIONS: Young children with severe TBI are at risk for severe, persisting attentional impairments. Children with mild and moderate TBI show better outcome, with pre-injury behaviour and age also predictive of attentional skills at 30 months post-injury.

Adaptation, Psychological↗

Ascaridole-less infusions of Chenopodium ambrosioides contain a nematocide(s) that is(are) not toxic to mammalian smooth muscle.

Infusions of Chenopodium ambrosioides (L.) have been used for centuries in the Americas as a popular remedy against intestinal worm infections. The essential oil of Chenopodium ambrosioides contains high levels of ascaridole, which is a potent anthelmintic, but which has also been responsible for human fatalities, leading to its disuse. Almost 90% of the nematocidal activity of Chenopodium ambrosioides infusions was due to a hydrophilic component different from ascaridole. Synthetic ascaridole and the ascaridole from infusions, extracted into hexane, caused a reduction of carbachol-induced contractions in rat gastrointestinal smooth muscle at concentrations required to kill Caenorhabditis elegans (L.). The herbal infusion and the ascaridole-free hexane-extracted aqueous residue of the above infusion, at nematocidal concentrations, had no detectable effect on smooth muscle contraction in the above system. It would appear that the traditional form of usage of Chenopodium ambrosioides infusions as a vermifuge is safer than the use of the herb's essential oil.

Animals↗

A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis.

OBJECTIVE: To determine if long-term topiramate therapy is safe and slows disease progression in patients with ALS. METHODS: A double-blind, placebo-controlled, multicenter randomized clinical trial was conducted. Participants with ALS (n = 296) were randomized (2:1) to receive topiramate (maximum tolerated dose up to 800 mg/day) or placebo for 12 months. The primary outcome measure was the rate of change in upper extremity motor function as measured by the maximum voluntary isometric contraction (MVIC) strength of eight arm muscle groups. Secondary endpoints included safety and the rate of decline of forced vital capacity (FVC), grip strength, ALS functional rating scale (ALSFRS), and survival. RESULTS: Patients treated with topiramate showed a faster decrease in arm strength (33.3%) during 12 months (0.0997 vs 0.0748 unit decline/month, p = 0.012). Topiramate did not significantly alter the decline in FVC and ALSFRS or affect survival. Topiramate was associated with an increased frequency of anorexia, depression, diarrhea, ecchymosis, nausea, kidney calculus, paresthesia, taste perversion, thinking abnormalities, weight loss, and abnormal blood clotting (pulmonary embolism and deep venous thrombosis). CONCLUSIONS: At the dose studied, topiramate did not have a beneficial effect for patients with ALS. High-dose topiramate treatment was associated with a faster rate of decline in muscle strength as measured by MVIC and with an increased risk for several adverse events in patients with ALS. Given the lack of efficacy and large number of adverse effects, further studies of topiramate at a dose of 800 mg or maximum tolerated dose up to 800 mg/day are not warranted.

Adult↗

Percutaneous endoscopic gastrostomy (PEG) in patients with ALS and bulbar dysfunction.

OBJECTIVE: To compare characteristics of ALS patients with and without percutaneous endoscopic gastrostomy (PEG). METHODS: Using the ALS Patient Care Database, data from patients with and without PEG with ALS Functional Rating Scale-bulbar subscale (ALSFRSb) scores < or = 5 were analyzed; follow-up data were also collected. RESULTS: PEG use was markedly increased with declining ALSFRSb scores. Demographics did not differ, but ALSFRS composite scores and bulbar and arm subscale scores were lower (P<0.0001). PEG patients used significantly more assistive devices, multidisciplinary care, home care nurses and aides, had more frequent physician and emergency department visits and hospital admissions (P<0.0001), and had lower health status based on the mini-SIP scale (P=0.0047). PEG use varied greatly between ALS centers. In the follow-up study, positive impact of PEG was noted in 79 % of PEG patients but in only 37.5% of patients who received PEG later, based on a small number of patients. PEG use showed no survival benefit. CONCLUSION: Patients did not receive PEG until bulbar function was severely reduced and overall ALS had markedly progressed. PEG may have been performed too late to demonstrate survival benefits. Aggressive proactive nutritional management appears essential in patients with ALS. To determine whether PEG provides benefits, it must be performed at earlier stages of the disease and prospectively studied.

Amyotrophic Lateral Sclerosis↗

Ethnicity and attitude toward menopause and hormone replacement therapy in Northern Israel.

PURPOSE: The aim of the study was to assess the knowledge, perception and attitude towards menopause and hormone replacement therapy (HRT) among women of different ethnic groups in Israel. METHODS: 292 postmenopausal women attending primary health-care centers in northern Israel were recruited. All women completed a questionnaire, which included information on demographics, health status, menopausal symptoms, attitude toward menopause, adequacy of counseling and experience with HRT. RESULTS: Thirty-five percent of women were Arabic women. Most of the population categorized menopause as a positive and natural event, only 26% of the Arabic women thought of menopause as an illness-oriented event compared to 7.7% of the Jewish women. Half the Arabic women had never received information or been counseled about menopause and HRT, compared to 18% of the Jewish women. Menopausal symptoms showed a statistically significant improvement with HRT. However, only 40% of the Jewish women and 24% of the Arabic women used HRT. CONCLUSIONS: Only a relatively small percentage of Arabic women know about menopause and HRT use, probably because of the relatively low rate of counseling and information provided about menopause and HRT, and their lack of knowledge. Greater efforts are needed to promote a positive attitude to aging and menopause, increase awareness and knowledge. Providing greater access to information and support are essential steps in improving the women's health of the Arabic population in Israel during menopausal years.

Arabs↗

A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients.

There is still no consensus as to which physiologic marker should be used as a trigger for the initiation of non-invasive positive pressure ventilation (NPPV) in patients with amyotrophic lateral sclerosis (ALS). Current practice parameters recommend that the decision to begin treatment be based upon forced vital capacity (FVC) measurements. A prospective, randomized study was performed in 20 ALS patients who had an FVC of 70-100%. Patients received baseline assessments including: ALS functional rating scale-respiratory version (ALSFRS-R), pulmonary symptom scale, Short form 36 (SF-36), FVC%, maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and nocturnal oximetry. Patients were randomized to receive NPPV based upon nocturnal oximetry studies suggesting oxygen desaturation <90% for one cumulative minute ("early intervention") or a FVC <50% ("standard of care"). At enrollment, there was no significant correlation between FVC% and the ALSFRS-R, symptom score, MEP, MIP, or duration of nocturnal desaturation <90%. An increase in the vitality subscale of the SF-36 was demonstrated in 5/6 patients randomized to "early intervention" with NPPV. Our data indicate that FVC% correlates poorly with respiratory symptoms and suggests that MIP and nocturnal oximetry may be more sensitive measures of early respiratory insufficiency. In addition, intervention with NPPV earlier than our current standard of care may result in improved quality of life.

Disease Progression↗

A randomized trial of very early decompressive craniectomy in children with traumatic brain injury and sustained intracranial hypertension.

OBJECT: The object of our study was to determine, in children with traumatic brain injury and sustained intracranial hypertension, whether very early decompressive craniectomy improves control of intracranial hypertension and longterm function and quality of life. METHODS: All children were managed from admission onward according to a standardized protocol for head injury management. Children with raised intracranial pressure (ICP) were randomized to standardized management alone or standardized management plus cerebral decompression. A decompressive bitemporal craniectomy was performed at a median of 19.2 h (range 7.3-29.3 h) from the time of injury. ICP was recorded hourly via an intraventricular catheter. Compared with the ICP before randomization, the mean ICP was 3.69 mmHg lower in the 48 h after randomization in the control group, and 8.98 mmHg lower in the 48 hours after craniectomy in the decompression group (P=0.057). Outcome was assessed 6 months after injury using a modification of the Glasgow Outcome Score (GOS) and the Health State Utility Index (Mark 1). Two (14%) of the 14 children in the control group were normal or had a mild disability after 6 months, compared with 7 (54%) of the 13 children in the decompression group. Our conclusion was that when children with traumatic brain injury and sustained intracranial hypertension are treated with a combination of very early decompressive craniectomy and conventional medical management, it is more likely that ICP will be reduced, fewer episodes of intracranial hypertension will occur, and functional outcome and quality of life may be better than in children treated with medical management alone (P=0.046; owing to multiple significance testing P <0.0221 is required for statistical significance). This pilot study suggests that very early decompressive craniectomy may be indicated in the treatment of traumatic brain injury.

Brain Injuries↗

Benzyl isothiocyanate is the chief or sole anthelmintic in papaya seed extracts.

Papaya (Carica papaya) seeds were extracted in an aqueous buffer or in organic solvents, fractionated by chromatography on silica and aliquots tested for anthelmintic activity by viability assays using Caenorhabditis elegans. For all preparations and fractions tested, anthelmintic activity and benzyl isothiocyanate content correlated positively. Aqueous extracts prepared from heat-treated seeds had no anthelmintic activity or benzyl isothiocyanate content although both appeared when these extracts were incubated with a myrosinase-containing fraction prepared from papaya seeds. A 10 h incubation of crude seed extracts at room temperature led to a decrease in anthelmintic activity and fractionated samples showed a lower benzyl isothiocyanate content relative to non-incubated controls. Benzyl thiocyanate, benzyl cyanide, and benzonitrile were not detected in any preparations and cyanogenic glucosides. which were present, could not account for the anthelmintic activity detected. Thus, our results are best explained if benzyl isothiocyanate is the predominant or sole anthelmintic agent in papaya seed extracts regardless of how seeds are extracted.

Animals↗

Two dimensional non equilibrium pH gel electrophoresis mapping of cytosolic protein changes caused by dietary protein depletion in mouse liver.

Two-dimensional non-equilibrium pH gel electrophoresis (2D-NEPHGE) analysis was used to evaluate the effects of dietary protein depletion on the protein composition of mouse liver cytosol. Analysing the cytosol from both normal and protein depleted liver, the position in gels of more than three hundred protein spots was determined. After 5 days of protein depletion, about 20% of the spots either increased or decreased more than 2 fold. Five spots of glyceraldehyde-3-phosphate dehydrogenase (GAPDH) were recognised by specific antibodies. The glutathione S-transferase (GSTs) subunits Ybl, Yc and Yf were identified by the simultaneous analysis of both glutathione-binding cytosolic proteins and the corresponding standards. As estimated by internal optical density (IOD) of spots, the changes caused by protein depletion in GAPDH and GST subunit contents were similar to those obtained by other methods. By means of mass spectrometric analysis of tryptic peptides generated from spots and/or comparison of two-dimensional gel electrophoretic patterns, carbonic anhydrase III (CAIII), Cu, Zn superoxide dismutase (CuZnSOD) and a cytochrome P450 cytosolic protein (cyt P450) were identified. These three proteins, as well as GSTs, are related with intracellular detoxification and free radical scavenging systems. Their contents were regulated by dietary protein restriction in a manner indicative of diminished liver defence against oxidising agents.

Animals↗

Outcome from mild head injury in young children: a prospective study.

There is a lack of agreement regarding the long-term consequences of mild head injury (HI) at any age, with such effects rarely studied in early childhood. Given the rapid development occurring within the brain during this period, any disruption may have the potential to cause transient or permanent damage to brain structure and function. The present study sought to investigate the behavioral implications of such potential disruptions using a prospective, longitudinal design. Children aged 3-7 years at the time of injury, and suffering from mild HI, were evaluated acutely and at 6 and 30 months post-injury. Pre-injury data were collected with respect to communication, social skills, daily living skills and behavioral function. Results were compared to those from a non-injured control sample matched for age, gender, socioeconomic status, and pre-injury function. Findings showed few group differences. Children with mild HI performed similarly to controls on measures of intellectual ability, receptive language, and both everyday and spatial memory capacity. Group differences were identified for verbal fluency and story recall, with HI children failing to recover over time.

Activities of Daily Living↗

Predictors of acute child and family outcome following traumatic brain injury in children.

OBJECTIVE: To examine the relative contributions of injury severity, level of physical and cognitive disability, child behavior and family function to short-term outcome 6 months following traumatic brain injury (TBI) in children. DESIGN: Prospective, longitudinal, between-group design, comparing preinjury and postinjury measures of functional outcome across three levels of injury severity. SUBJECTS: One hundred and twelve children, aged 2-12 years admitted to the Royal Children's Hospital, Melbourne, with a diagnosis of TBI. The sample was divided into three groups, according to injury severity: mild TBI (n = 31), moderate TBI (n = 52) and severe TBI (n = 29). Children with a history of neurologic, developmental and psychiatric disorder were excluded from participation. MAIN OUTCOME MEASURES: Levels of postinjury functioning in the following domains: physical function, cognitive ability (incorporating intellect, memory and attention), behavioral and family functioning, and level of family burden. RESULTS: A clear dose-response relationship for physical and cognitive outcomes was found, with severe TBI associated with greater impairment of physical, intellectual, memory and attentional function. For psychosocial outcome, results were less clearly linked to injury severity. Preinjury behavioral and family functioning were closely related to postinjury function in these domains, with an interaction identified between family function and child behavior at 6 months postinjury. Family functioning remained unchanged postinjury, although level of burden was high, and predicted by injury severity, functional impairment and postinjury behavioral disturbance. CONCLUSIONS: These results suggest ongoing functional problems for the child and significant family burden 6 months following TBI. The nature and severity of physical and cognitive problems are most closely related to injury severity, with family functioning and child behavior better predicted by psychosocial and premorbid factors.

Acute Disease↗

Motor neuron disease.

Motor neuron disease refers to a spectrum of disorders resulting from degeneration of the upper or lower motor neurons or both. Amyotrophic lateral sclerosis is the most common form of motor neuron disease, in which patients demonstrate evidence of both anterior horn cell (lower motor neuron) and corticospinal tract (upper motor neuron) dysfunction. Several theories regarding the pathogenesis of amyotrophic lateral sclerosis have emerged, including glutamate excitotoxicity, free radical oxidative stress, cytoskeletal abnormalities, a deficiency of neurotrophic factor, autoimmunity, apoptosis, and viral infection. Numerous clinical trials have been completed based on these possible mechanisms of the disease propagation including treatment with antiglutamate agents, anti-oxidants, immunosuppressants, and neurotrophic factors. Several of these trials have shown modest effects in slowing the disease course. None, however, have yielded marked benefit in arresting disease progression. The most significant effect in abating disease progression has been our use and understanding of aggressive symptomatic therapy to reduce disability, enhance quality of life, and improve prognosis.

Amyotrophic Lateral Sclerosis↗

[Invasive aspergillosis of the orbit in immunocompetence].

BACKGROUND: An isolated aspergillosis of the sphenoidal sinus is a difficult diagnosis because the often misleading clinical symptoms of this rare disease can develop late and first be presented to the ophthalmologist. Because of this even life-threatening disease in normally immunodeficient patients, fast diagnostics and treatment is indicated. CASE REPORT: We report about an immunocompetent man to suffer from an invasive aspergillosis involving the sphenoidal sinus after external operation of a mucocele. Aspergillus infiltrated the right orbit by bone erosion and led to an acute loss of vision on his right eye and a pathological visual field (visual acuity 0.1). A systemical antimycotic therapy was performed and the right orbit and the optical channel were operated to remove the mycosis. During operation the optical nerve was found to be completely surrounded by the mycosis. After surgery visual acuity recovered slowly but completely in about two weeks (visual acuity 1.0) and visual field examination revealed normal findings. These findings were stable now for one year. CONCLUSION: The main findings of unilateral acute visual loss and pre-operations of the paranasal sinuses in combination with persistent headache can lead to the suspect of an aspergillosis infection. It is important to establish correct diagnosis--especially in immunocompetent patients--early to prevent a probably irreversible visual loss and in an advanced stage to handle the life-threatening risk of this disease.

Aged↗

Recovery of memory function following traumatic brain injury in pre-school children.

Traumatic brain injury (TBI) may have a profound impact on a child's ongoing development. Various risk factors have been found to predict outcome, but considerable variability remains unexplained. This study used a prospective, longitudinal design to examine recovery of memory function following TBI within the pre school period. Ninety-six children with TBI were divided according to injury severity (mild, moderate, severe), and compared to age and SES matched healthy controls (n = 35). Children were evaluated acutely and at 6,12 and 18 months post-injury using intellectual and memory measures. Results showed a relationship between greater injury severity and poorer intellectual ability. This dose-response relationship was not clearly evident for memory function within the acute phase of recovery, but developed over time, with greater memory impairments evident for children with more severe TBI by 12 months post-injury. Children with mild TBI exhibited few memory problems. Findings are discussed in the context of theories of plasticity and recovery of function.

Amnesia↗