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Biomedical subjects

C M Duffy

Publications and source records attributed to C M Duffy.

32 records · Page 2Linked to original sources
PubMed1995

Juvenile spondyloarthropathies: clinical manifestations and medical imaging.

The spondyloarthropathies comprise four distinct entities--ankylosing spondylitis, psoriatic arthritis, the arthritis associated with inflammatory bowel disease, and Reiter's syndrome and other related forms of reactive arthritis. Although these are distinct diseases, they have a number of clinical, radiologic, and genetic characteristics in common which permit them to be classified under the unifying term "spondyloarthropathy". They are diseases of young adults, and when they present in patients under 16 years of age we refer to them as the "juvenile" spondyloarthropathies. They must be distinguished from juvenile rheumatoid arthritis, which is a totally separate entity; however the distinction may not always be obvious. Involvement of peripheral and sacroiliac joints commonly occurs in the juvenile spondyloarthropathies. The peripheral arthritis may be erosive and associated with bone apposition at the joint margins. Axial involvement is usually a late finding. Dactylitis and tenosynovitis are frequently present early on. Enthesitis, a highly specific feature, occurs much more often in the juvenile spondyloarthropathies than in the adult forms and it may be the only presenting feature. The plain radiograph is the primary and most important imaging modality for the assessment of these diseases. However, an expanding role of magnetic resonance imaging is evident.

Adolescent↗
PubMed1994

Incorporation of Rap 1b into the platelet cytoskeleton is dependent on thrombin activation and extracellular calcium.

Rap 1b is a 22-kDa low molecular mass GTP-binding protein which is both a member of the Ras superfamily and a substrate for cAMP-dependent protein kinase. Recently, evidence has been presented to show that Rap 1b is incorporated into the detergent-extracted cytoskeleton of platelets during thrombin-induced activation. The aims of this study were to compare the incorporation of Rap 1b into the detergent-extracted cytoskeleton after activation with different agonists, to examine the role of extracellular calcium on the incorporation of Rap 1b into the cytoskeleton, to investigate the relationship between the association of Rap 1b and other proteins with the cytoskeleton, and to determine the effect of phosphorylation of Rap 1b incorporation into the cytoskeleton. Platelets were activated with thrombin, A23187, phorbol myristate acetate, ADP, epinephrine, and collagen in the presence and absence of calcium. The time dependence of Rap 1b incorporation into the detergent-extracted cytoskeleton was then measured. When platelets were activated by thrombin in the presence of extracellular calcium, conditions which permit aggregation, incorporation of Rap 1b into the detergent-extracted cytoskeleton was biphasic. Approximately 20% of the total cellular Rap 1b incorporated into the cytoskeleton within seconds and was followed by a slower second phase of incorporation. In contrast, when platelets were activated by thrombin in the absence of calcium, conditions which inhibit aggregation, or by the other agents in the presence or absence of calcium, only the initial phase of Rap 1b incorporation into the cytoskeleton was measured. The incorporation of Rap 1b paralleled the incorporation of membrane glycoproteins (GP) IIb/IIIa and PECAM-1, but not the incorporation of pp60c-src. The GTPase-activating protein for Ras (Ras-GAP) did not associate with the detergent-extracted cytoskeleton. Two-dimensional isoelectric focusing SDS-polyacrylamide gel electrophoresis of the total cellular and cytoskeletal Rap 1b showed that unphosphorylated as well as phosphorylated isoforms of Rap 1b were incorporated into the cytoskeleton in the same molar ratio as was present in the intact cell. Furthermore, the rates of incorporation of phosphorylated and unphosphorylated Rap 1b into the cytoskeleton were similar. These experiments show that Rap 1b can regulate events that take place within seconds after activation, such as the initial formation of the cytoskeleton, as well as longer term changes in the cytoskeleton that occur in response to thrombin-induced aggregation. Furthermore, phosphorylation could modulate the (unknown) functions of Rap 1b as a component of the cytoskeleton.

Blood Platelets↗
PubMed1993

Level of agreement between parents and children in rating dysfunction in juvenile rheumatoid arthritis and juvenile spondyloarthritides.

OBJECTIVE: To determine the level of agreement between parents and children in rating dysfunction in juvenile rheumatoid arthritis (JRA) and juvenile spondyloarthritides. METHODS: We conducted separate interviews (parents and patients) of children > or = 9 years of age with JRA and spondyloarthritis attending our clinic. A questionnaire consisting of 100 probes (57 physical activities, 26 psychosocial issues and 17 general symptoms) was used. Probes were scored by respondents on a 6-point scale for their frequency of occurrence (all probes) and importance to the patient (physical activities, only), giving a total of 157 scores. Forty patients were included. Level of agreement between patients and parents was determined by weighted kappa (kappa). RESULTS: Mean kappa for all scores was 0.60; gross motor 0.51, fine motor 0.64, psychosocial 0.56 and general symptoms 0.64. One hundred and forty scores (89%) showed at least moderate agreement. CONCLUSIONS: Our data confirm that there is good parent-child agreement on the level of dysfunction of children > or = 9 years of age with JRA and spondyloarthritis. This level of agreement extends over a wide range of general symptoms and includes physical and psychosocial function. Thus both the parent and the child are reliable informants on the impact of chronic arthritis on the quality of life of affected children and either can be used for this type of assessment.

Adolescent↗
PubMed1992

The utility of the arthritis impact measurement scales for patients with psoriatic arthritis.

The Arthritis Impact Measurement Scales (AIMS) consists of 9 scales that measure physical function, pain and psychosocial function. It has been validated for use in various forms of arthritis, but not in psoriatic arthritis (PsA). The AIMS was administered to 145 patients attending our PsA clinic. We carried out simultaneous assessment of clinical measures of function, measures of disease activity, and measures of disease severity. Most scales of physical function were moderately to highly correlated with clinical measures of function (r = 0.33-0.57; p = 0.0001), measures of disease activity (r = 0.24-0.53, p = 0.003-0.0001), and measures of disease severity (r = 0.23-0.6; p = 0.02-0.0001). The pain scale was highly correlated with clinical measures of function and measures of disease activity (r = 0.38-0.58; p = 0.0001) but not with measures of disease severity. Of the psychosocial scales, the depression scale was moderately correlated with clinical measures of function (r = 0.27-0.3; p = 0.001-0.0001). Our data suggest that the physical function and pain scales are good indicators of overall function and disease activity and are valid for use in PsA.

Activities of Daily Living↗
PubMed1991

Infection and disease activity in systemic lupus erythematosus: a review of hospitalized patients.

Infection is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). A review of all SLE admissions to our hospital during a 5-year period was conducted to determine the rate and nature of infection, and its association with overall disease activity, measured by the SLE Disease Activity Index (SLEDAI). Eighty-one patients (79 women, 2 men) were admitted for a total of 2,738 days (176 admissions). There were 53 proven infections, giving an infection rate of 1.94/100 hospital days. Twenty-three (43.4%) of these were major infections (requiring IV antibiotics). Two of 3 deaths were due to septicemia. By logistic regression analysis, infection was significantly associated with disease activity (p = 0.005), but not with disease duration or prednisone dosage. Our data confirm that infection is common in hospitalized patients with SLE, is associated with overall disease activity independent of prednisone dose, and causes significant mortality. These facts should be borne in mind when hospitalization is considered for patients with SLE.

Adult↗
PubMed1990

Home-based palliative care for children--Part 2: The benefits of an established program.

Thirty-four patients have been admitted to our palliative care program since its institution in March 1986. Five were unsuitable and were withdrawn soon after admission. Of the remainder, 22 (75.9%) had central nervous system (CNS) tumors, 5 (17.2%) had myelomeningocoele, 1 (3.45%) had an arteriovenous (AV) malformation, and 1 (3.45%) had a storage disease. Twenty-five (86.2%) have since died and 17 (68%) of these have died at home. In comparison with a similar group of 30 patients studied in a feasibility study prior to the institution of our program, patients admitted to our palliative care program were hospitalized for significantly fewer days during the terminal phase of their illness (p less than 0.05) and a significantly higher proportion died at home (p less than 0.001). Following a survey of the parents of 14 children diagnosed with CNS tumors who died while under our care, we determined that the overall level of satisfaction with the program was high compared to care provided prior to admission to the program (p less than 0.01). Components of the program deemed most satisfactory were (1) being able to care for the child at home (p less than 0.01), (2) having access to a palliative care nurse (p less than 0.05), and (3) having access to a pediatric clinical pharmacologist (p less than 0.05). The most troublesome symptoms occurring in this group of patients were pain, gastrointestinal symptoms, and seizures. Most problems were adequately managed by the parents under the supervision of the team. Eighty percent of the terminal care for these patients was provided at home.(ABSTRACT TRUNCATED AT 250 WORDS)

Central Nervous System Diseases↗
PubMed1990

Home-based palliative care for children--Part 1: The institution of a program.

To assess the necessity and feasibility of introducing a home-based palliative care program for children admitted to our neurosurgical unit and diagnosed as being terminally ill, we conducted an analysis of all such patients admitted to our unit over an 18-month period. Of a total of 30 patients, 22 (73.4%) had central nervous system tumors, 6 (20.0%) had myelomeningocele/hydrocephalus, and 2 (6.6%) had arteriovenous malformations. The mean duration +/- SEM of hospitalized days prior to death and the proportion of time spent hospitalized during the terminal phase of illness were 28.8 +/- 4.77 and 0.327 respectively, for the 23 patients who died and on whom adequate data were available. Sixteen (70.6%) of these 23 patients died in hospital. Nine (30.0%) of the total group of patients were studied prospectively during the last 6 months of this study to determine the extent of their symptoms and to ascertain whether it would be feasible for them to be managed at home. The most troublesome symptoms in this subgroup were feeding difficulties, gastrointestinal symptoms, breathing difficulty, and seizures. These symptoms were managed either by (a) medications administered orally or by feeding tube or rectally, or by (b) noninvasive procedures carried out by a nurse under the direction of the admitting neurosurgeon. This study suggests that prolonged hospitalization for children diagnosed as being terminally ill can be avoided by introducing a home-based palliative program with involvement from a nurse and a physician familiar with drug therapy for terminally ill children.

Adolescent↗
PubMed1989

Raynaud syndrome in childhood.

Twenty-seven patients with Raynaud syndrome (mean age at onset 11.7 years) were studied to determine the prevalence of primary Raynaud syndrome and to assess the predictive role of antinuclear antibody, nail-fold capillary microscopy, and photoelectric plethysmography in this population. Fourteen patients (52%) had a connective tissue disease, four (15%) had a probable connective tissue disease, and nine (33%) had primary Raynaud syndrome. In all patients with either a connective tissue disease or a probable connective tissue disease, there was a positive reaction to antinuclear antibody, in contrast to patients with primary Raynaud syndrome, in whom antinuclear antibody was not detected. Nail-fold capillary microscopy scores differed significantly between patients with either a connective tissue disease or a probable connective tissue disease and those with primary Raynaud syndrome for both enlarged loop score (p less than 0.025 and less than 0.05, respectively) and avascular score (p less than 0.005 and less than 0.01, respectively). Photoelectric plethysmography scores were reduced in all groups but did not differ significantly between groups. Our findings suggest that in children with Raynaud syndrome, the primary type is more common than was originally suspected, and that both antinuclear antibody and nail-fold capillary microscopy, but not photoelectric plethysmography, can distinguish patients with primary Raynaud syndrome from those with either a connective tissue disease or a probable connective tissue disease.

Adolescent↗
PubMed1989

Drug therapy for juvenile arthritis.

Juvenile arthritis includes a variety of rheumatic disorders with varied articular and extraarticular manifestations. The heterogeneity of JA cannot be overemphasized in a rational approach to therapy. Fortunately, most children with JA improve significantly with first-line treatment, especially those with pauciarticular disease. Only a small number of these patients go on to require second-line treatment, usually because of the evolution of their condition into the polyarticular type. Patients with polyarticular, RF-positive disease and patients with early-onset polyarthritis in association with systemic-onset disease both have poor prognoses in terms of ultimate joint function, and should receive second-line treatment early in their therapy. Injectable gold is the drug of choice for the former patients, if an initial trial of NSAIDs has failed. Patients with polyarticular onset, RF-negative disease generally have a better prognosis than other patients with polyarticular involvement. One may wait considerably longer before introducing a second-line agent for these patients. An antimalarial agent may be a reasonable choice for these patients, especially those with limited polyarticular involvement (5 to 10 active joints). This latter principle may also be applied to patients who evolve from pauciarticular to polyarticular involvement. In all such cases, penicillamine is almost never used as first choice. If the patient is a young child, the difficulty with injecting gold and the hazards of antimalarial agents may limit the use of these treatments. In situations such as this, auranofin or sulfasalazine may be the drug of choice. Cytotoxic/antimetabolic therapy should be reserved for those children who have continued active disease despite full courses of two SAARDs, or for those who have been unable to take SAARDs because of their side effects. Corticosteroid therapy should be used only when specifically indicated, and every attempt should be made to wean the patient from it as soon as feasible. The aim of therapy is to ensure that patients enter their remission in the best condition possible. For this to be assured, a sensible approach to drug therapy is mandatory.

Adolescent↗
PubMed1984

General practitioners' use of a mobile coronary care unit in a non-urban setting.

The use that general practitioners made of the mobile coronary care unit operating from a district general hospital was monitored. Over half of the patients referred by their general practitioners to hospital with suspected acute myocardial infarction were removed by the mobile unit rather than by an ordinary ambulance. However, general practitioner selection of the more compromised patient for admission via the mobile unit indicated that the chance of this category of patient being admitted by the proven best means could be improved. Of the definite cases of infarcts removed to hospital by whatever means, the high proportion which were uncomplicated raises the question whether the general practitioner is attempting to select such patients for home rather than hospital care. The figures for the use of the mobile coronary care unit also indicate that education of the public remains an important task.

Coronary Care Units↗
PubMedDate not supplied

Three-dimensional gait analysis in spina bifida.

This study was designed to determine gait patterns in children with lumbar and sacral neurologic level spina bifida. We studied a group of 28 children: 10 had L4-level lesions and a mean age of 11 years; eight had L5-level lesions and a mean age of 8 years; and 10 had S1-level lesions with a mean age of 12 years. A group of 15 normal children, mean age 10 years, was used for comparison. Each child underwent three-dimensional gait analysis using the Vicon system. We found that there were recognisable gait patterns for each level of spina bifida and that the abnormalities accurately reflected the muscle deficiencies present. The gait patterns approximated more closely to those of the normal group as the neurological level descended. The most important findings were of increased pelvic obliquity and rotation with hip abduction in stance (reflecting the gross Trendelenburg-type gait seen in these children) and persistent knee flexion throughout stance as a result of the absence of the plantar flexion-knee extension couple. We found that gait was not improved by tendon transfers performed either at the hip (posterolateral psoas transfer) or at the ankle (tibialis anterior transfer).

Adolescent↗
PubMedDate not supplied

The influence of ankle-foot orthoses on gait and energy expenditure in spina bifida.

We examined the effect of ankle-foot orthoses (AFOs) on gait and energy expenditure in children with spina bifida. Nine boys and three girls, 6 to 16 years of age, took part in the study. There were four children each with L4, L5, and sacral level lesions. Each child underwent gait analysis and energy consumption studies with and without AFOs. Walking speed was faster with AFOs (mean, 58 m/min) than without (mean, 50 m/min, p < 0.01). Stride length improved significantly (p < 0.001) from 0.89 m barefoot to 1.08 m with AFOs. Double support time was decreased from 0.32 seconds barefoot to 0.28 seconds with AFOs (p < 0.05). The oxygen cost of walking was significantly better with (0.33 mL/kg/m) than without AFOs (0.41 mL/kg/m, p < 0.001). Hip flexion at initial contact was increased. Ankle kinematics were unchanged, but ankle power generation showed a significant improvement; from 0.5 W/kg barefoot to 1.3 W/kg with AFOs. Increased ankle power generation results in improved hip flexion and stride length, which contributes to increased walking speed and reduced oxygen cost. The stability conferred by the AFOs is reflected in the reduced time spent in double support.

Adolescent↗
PubMedDate not supplied

Botulinum toxin A compared with stretching casts in the treatment of spastic equinus: a randomised prospective trial.

Conservative therapies for equinus in cerebral palsy may help to postpone calf surgery in younger children. This study reports a prospective randomised trial of intramuscular botulinum toxin A (BtA) as an alternative to serial casting in 20 children with a dynamic component to calf equinus. Outcome was assessed in the short term to show the effect of one treatment cycle. Assessments were by clinical examination, video gait analysis, and three-dimensional gait analysis. BtA was of efficacy similar to that of serial casting. Tone reduction in the BtA group allowed a more prolonged improvement in passive dorsiflexion, which may allow more opportunity for increase in muscle length. Gait analysis showed an improved mean ankle kinematic pattern in a subsection of both groups, which was maintained at 12 weeks in the BtA group, whereas the cast group relapsed. There were fewer side effects in the BtA group. Median time to reintervention was similar.

Botulinum Toxins, Type A↗