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Interobserver reliability of rheumatologists performing musculoskeletal ultrasonography: results from a EULAR "Train the trainers" course.

OBJECTIVE: To evaluate the interobserver reliability among 14 experts in musculoskeletal ultrasonography (US) and to determine the overall agreement about the US results compared with magnetic resonance imaging (MRI), which served as the imaging "gold standard". METHODS: The clinically dominant joint regions (shoulder, knee, ankle/toe, wrist/finger) of four patients with inflammatory rheumatic diseases were ultrasonographically examined by 14 experts. US results were compared with MRI. Overall agreements, sensitivities, specificities, and interobserver reliabilities were assessed. RESULTS: Taking an agreement in US examination of 10 out of 14 experts into account, the overall kappa for all examined joints was 0.76. Calculations for each joint region showed high kappa values for the knee (1), moderate values for the shoulder (0.76) and hand/finger (0.59), and low agreement for ankle/toe joints (0.28). kappa Values for bone lesions, bursitis, and tendon tears were high (kappa = 1). Relatively good agreement for most US findings, compared with MRI, was found for the shoulder (overall agreement 81%, sensitivity 76%, specificity 89%) and knee joint (overall agreement 88%, sensitivity 91%, specificity 88%). Sensitivities were lower for wrist/finger (overall agreement 73%, sensitivity 66%, specificity 88%) and ankle/toe joints (overall agreement 82%, sensitivity 61%, specificity 92%). CONCLUSION: Interobserver reliabilities, sensitivities, and specificities in comparison with MRI were moderate to good. Further standardisation of US scanning techniques and definitions of different pathological US lesions are necessary to increase the interobserver agreement in musculoskeletal US.

Adult↗

Time of day of prednisolone administration in rheumatoid arthritis.

Twelve patients with rheumatoid arthritis took low dosage prednisolone, mean 5.6 mg daily, at either 0800 h, 1300 h or 2300 h in a double-blind within-patient controlled trial. Each patient was studied on each of the 3 regimens to assess control of symptoms and side effects and also to examine circadian rhythms in signs and symptoms. For several days during each drug regimen patients collected urine at each micturition and self-assessed their signs and symptoms. Circadian rhythms of finger joint swelling and of grip strength were determined, and were similar on all regimens, with morning peaks of symptoms and signs. Subjective and objective assessments showed no differences in effectiveness between the 3 times of administration of prednisolone. Urinary excretion patterns were similar to those observed in untreated people. The quantity and circadian pattern of 11-hydroxycorticosteroids excreted were similar to those in healthy patients, providing no evidence of adrenal cortical suppression at the dose levels studied, even when this dose was taken in the evening. A single morning dose of prednisolone appears in many patients to be as effective as a single evening dose or divided doses. It is therefore reasonable to initiate therapy with a morning-only regimen, because adrenopituitary suppression should be minimised.

11-Hydroxycorticosteroids↗

Radiographic changes in the hands of rock climbers.

Radiographs of the hands of 36 rock climbers were compared with radiographs of the hands of controls matched for age and sex. Subchondral cysts were present in the hands of 17 climbers and only two controls. Osteophytes or bony spurs were present in 14 climbers but not in any of the controls, and the only two cases of frank osteoarthrosis were in the hands of climbers. Cortical thickness of the proximal and middle phalanges was significantly greater in the hands of climbers (P = < 0.01). Pronounced 'scalloping' of the necks of the proximal phalanges was only seen in climbers, and is due to thickening of the attachment of the distal end of the fibrous A2 pulley of the flexor sheath.

Adult↗

The phenotypic spectrum in patients with arginine to cysteine mutations in the COL2A1 gene.

BACKGROUND: The majority of COL2A1 missense mutations are substitutions of obligatory glycine residues in the triple helical domain. Only a few non-glycine missense mutations have been reported and among these, the arginine to cysteine substitutions predominate. OBJECTIVE: To investigate in more detail the phenotype resulting from arginine to cysteine mutations in the COL2A1 gene. METHODS: The clinical and radiographic phenotype of all patients in whom an arginine to cysteine mutation in the COL2A1 gene was identified in our laboratory, was studied and correlated with the abnormal genotype. The COL2A1 genotyping involved DHPLC analysis with subsequent sequencing of the abnormal fragments. RESULTS: Six different mutations (R75C, R365C, R519C, R704C, R789C, R1076C) were found in 11 unrelated probands. Each mutation resulted in a rather constant and site-specific phenotype, but a perinatally lethal disorder was never observed. Spondyloarthropathy with normal stature and no ocular involvement were features of patients with the R75C, R519C, or R1076C mutation. Short third and/or fourth toes was a distinguishing feature of the R75C mutation and brachydactyly with enlarged finger joints a key feature of the R1076C substitution. Stickler dysplasia with brachydactyly was observed in patients with the R704C mutation. The R365C and R789C mutations resulted in classic Stickler dysplasia and spondyloepiphyseal dysplasia congenita (SEDC), respectively. CONCLUSIONS: Arginine to cysteine mutations are rather infrequent COL2A1 mutations which cause a spectrum of phenotypes including classic SEDC and Stickler dysplasia, but also some unusual entities that have not yet been recognised and described as type II collagenopathies.

Adult↗

Clinical features and reproductive patterns in fragile X female heterozygotes.

Clinical findings are presented on 113 fragile X female heterozygotes from 44 families, based on physical examination, behavioural assessment, and reproductive history. In 85% of a subsample of 92 adult females non-verbal IQ score derived from the Block Design test was 85 or less. Verbal ability deficits were much less common. Typical facial characteristics, irregular teeth, and hypermobility of finger joints occurred in approximately 40% of adult females, but facial abnormalities were less common in children. Some physical anomalies and the level of intellectual impairment were, in adult carriers, associated with the presence of fragile X sites. The commoner physical anomalies or typical facial characteristics and intellectual abilities differed significantly between the known female heterozygotes and their 40 presumed normal relatives. Frequency of miscarriages was increased in fragile X females; in spite of this, a moderate increase in the number of children has been encountered in female carriers with borderline intellectual impairment. This important problem has genetical implications and needs further investigation. The importance is emphasised of a more detailed clinical examination of the females at risk in fragile X families.

Abortion, Spontaneous↗

Costello syndrome in two Brazilian children.

The increasing number of children with Costello syndrome described world wide has helped in delineating further the characteristic features of this condition. We report here two children, seen in the Genetic Division of the Brasilia University Hospital, showing the main features of the syndrome: "coarse" face, redundant skin on the feet and hands, hyperextensible hand and finger joints, curly hair, growth and psychomotor retardation, and feeding problems. In addition, one of the patients developed hydrocephalus during the evolution of the disease, making the third published patient with this complication. We discuss the appropriateness of serial brain imaging studies in Costello syndrome in light of the relative frequency of central nervous system malformations.

Brazil↗

Beats produced between a rhythmic applied force and the resting tremor of Parkinsonism.

Rhythmic forces have been applied to the wrist of patients with Parkinsonism tremor by means of a printed motor. The tremor rate was not altered to that of the applied force. On the contrary, beats were established, the rate of which depended on the difference in rate between the tremor and the applied rhythm. Most of the observations have been for horizontal motion of the hand but similar phenomena have been seen for vertical movements, and for other parts of the body--for example, foot, elbow, finger joint, and head. The observations are regarded as supporting the view that the tremorgenic mechanism is central. There was no electromyographic evidence of servo driving or servo assistance in the genesis of the tremor.

Electromyography↗

Functional anatomy of the flexor tendon system of the hand.

The beautifully coordinated functions of the hand depend on the tendon apparatus acting on the articulated skeletal elements. The flexor muscles are situated on the antero-medial aspect of the forearm, with their tendons crossing the wrist and finger joints in a wonderfully arranged system to provide the infinite movements possible. The anatomical details of the extrinsic and intrinsic flexor apparatus are presented, with their functions described as anticipated by their origins, insertions, and the joints they cross and thus can act upon.

Anatomy, Cross-Sectional↗

Flexor tendon sheath ganglion: analysis of 128 cases.

A 30-year analysis of 128 patients with flexor tendon sheath ganglion was investigated. The majority of patients were females with sex ratio of 2.6 : 1. Most of the patients are in their third to fifth decade of life. Hand dominance, previous trauma as well as other illnesses involving the hand did not show any correlation to the formation of ganglion. The middle finger was most commonly affected and 69% of the ganglion were located on A1 and A2 pulley. Recurrence was high (89%) after multiple percutaneous puncture and treatment was successful with no cases of recurrence after surgical excision.

Adolescent↗

The arthropathy of hemochromatosis.

Five cases of hemochromatosis arthropathy are presented and the distinctive radiological features of the disease are described. Although the condition is typically degenerative, showing subchondral cyst formation, sclerosis, and thinning of cartilage, its distribution is characteristic. Selective degenerative changes of the second and third metacarpophalangeal joints are striking, particularly in the hands, while abnormalities in the intercarpal joints are variable and the interphalangeal joints are spared. Chondrocalcinosis involving both fibrous and hyaline cartilage is frequently seen as well, particularly in the large joints. The calcification is due to deposition of calcium pyrophosphate crystals, perhaps resulting from iron inhibition of pyrophosphatase.

Adult↗

Trichorhinophalangeal syndrome.

Trichorhinophalangeal syndrome (TRPS) comprises a distinctive combination of hair, facial and bony abnormalities with variable expression. A 20-year-old man with TRPS was seen because of marked androgenetic alopecia. Scanning electron-microscopic studies of the hair revealed flattened hair with an elliptoid transverse section pattern. Mechanical behavior of the hair was abnormal with a significant increase in the viscous parameter, indicating a decreased intermolecular bridging within the keratin matrix. The dermatologist confronted with premature or marked alopecia in young adults should always consider the possibility of an underlying congenital syndrome involving the hair and prompt further investigation.

Adult↗

[Posner-Schlossman syndrome].

The clinical features of Posner-Schlossman syndrome and the various differential diagnoses are described in a 20-year-old patient. Additional to Posner-Schlossman syndrome this patient showed signs of Axenfeld's anomaly. He also presented symptoms of a status-dysrhaphicus-like state after atrial septal defect and the ability to overstrain the distal finger joints. Posner-Schlossman syndrome, heterochromic cyclitis, anterior-chamber cleavage syndrome and ICE syndrome (iridocorneal-endothelial syndrome: Chandler's, Cogan-Reese syndrome and progressive essential iris atrophy) often produce similar effects at the Descemet membrane, the anterior chamber angle and the iris. Because of the same mesodermal origin of these tissues we suggest that all the diseases mentioned above are merely different clinical expressions of one main disease.

Adult↗

Hand injury patterns in softball players using a 16 inch ball.

Softball is a popular recreational and competitive sport among both men and women. The injury rate in softball players is as high as that in baseball and basketball players. We conducted a retrospective analysis of 119 hand injuries in 108 patients treated at the University of Chicago hand clinic. All of the injuries were caused by the impact of a 16 inch circumference softball. Of the 119 injuries, 87 (73%) had bone involvement. Operative treatment was required in 26 (22%) injuries, 23 involving fractures and 3 involving soft tissue only. There was one (3.8%) operative complication. Of all injuries, 101 (86%) involved the finger joints, including 46 (39%) injuries to the distal interphalangeal (DIP) joint, 48 (40%) to the proximal interphalangeal (PIP) joint, and 7 (6%) to the metacarpophalangeal (MCP) joint. The most common DIP joint injury was a mallet injury. This fracture, the most common single type of injury in our series, accounted for 27% of all injuries. Of all mallet injuries, 86% were fractures. The most common PIP joint injury was a volar plate fracture, the second most common injury in our series. Variables such as the patient's sex, dominance or nondominance of hands, and early or late season play were not associated with a higher risk of injury. Certain parts of the hand, such as the more ulnar digits and the DIP and PIP joints, were at particularly high risk of injury.

Adolescent↗

Characterization of Hexsyn, a polyolefin rubber.

Hexsyn is the Goodyear Tire and Rubber Company tradename for a polyolefin rubber synthesized from 1-hexene with 3-5% methylhexadiene as the source of residual double bonds for vulcanization. Under license from Goodyear, this same polymer has been manufactured by Lord Corporation for the hinge portion of finger joint prostheses using the tradename Bion. This rubber is currently licensed to the University of Akron and to the Cleveland Clinic Foundation for use in biomedical applications, and is being used primarily for biocompatible and highly fatigue resistant rubber components in ventricular assist and artificial heart systems. Results are presented from the physical, mechanical, and biological characterization of Hexsyn. Procedures are described for the synthesis, compounding, and post-molding extraction for Hexsyn. The physical testing of Hexsyn reported includes determinations of its density at 23 and 37 degrees C, initial hardness and hardness after aging in oxygen, blood, pseudoextracellular fluid and polyethylene glycol 600, typical molecular weights determined by gel permeation chromatography/low angle laser light scattering and intrinsic viscosity, thermal analyses by differential scanning calorimetry of Hexsyn gum, and vulcanized Hexsyn after exposure to blood and blood/fatigue conditions. Also reported are results of differential thermal analyses, thermomechanical analyses of virgin and annealed samples, and thermogravimetric analyses conducted in helium and in air. Dynamic mechanical analyses of Hexsyn include Clash-Berg and Rheovibron tests. Swelling was conducted to determine lot-to-lot and sheet-to-sheet variation for quality control and also a number of solvents were used so that the polymer-solvent interaction parameters could be determined. The permeability of Hexsyn to water, water vapor, and a variety of gases is reported. The permeability by contact angle measurements, refractive index, residual solvent analyses, migration of blood components into Hexsyn, melt rheology by Monsanto Rheometer, resistance to acids, and typical mold shrinkage for Hexsyn are reported. Mechanical testing of Hexsyn includes tensile strength, elongation, and tensile stress (modulus) at 23 and 37 degrees C and after conditions including exposure to blood, pseudoextracellular fluid, polyethylene glycol, oxygen, 100% relative humidity, and fatigue testing. Stress/strain calibration curves, flexural rigidity after aging in blood, tension set, compression set, stress relaxation, and the effect of repeated cycling on the elastic modulus are presented along with the results of Pico abrasion, skid resistance tests on wet concrete.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

Finger osteoarthritis and differences in dental work tasks.

Mechanical load has been proposed as a risk factor for hand osteoarthritis. Dentists produce high manual forces in their work tasks. We studied whether the pattern of dental work tasks was associated with finger osteoarthritis. Radiographs of both hands of 291 middle-aged female dentists were examined for the presence of osteoarthritis. Patterns of dental work tasks during work history were empirically defined by cluster analysis. Three patterns emerged reflecting high, moderate, and mild task variation. Age, specialization, years in clinical work, various activities requiring hand use, family history of Heberden's nodes, body mass index, and smoking were accounted for in logistic regression analyses. The dentists with a history of low task variation had a greater prevalence of osteoarthritis in the thumb, index, and middle fingers compared with dentists with high variation (OR 2.22; 95%CI 1.04-4.91). The pattern of dental work task history is associated with the localization of osteoarthritis in the fingers.

Cluster Analysis↗