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Biomechanical factors affecting progression of structural scoliotic curves of the spine.

The development of the spine is affected by both biological and mechanical factors. As the geometry of the motion segment changes throughout growth, so does the mechanical behaviour of the spine owing to changing vectors acting on a variable geometry system. The biomechanical analysis of the growing spine enables the assessment of the role played by biological and mechanical factors in the pathogenesis of progressive scoliosis to be assessed and its comparison with factors acting on an adult scoliotic spine. The knowledge of these principles is instrumental to setting the right parameters for treatment and to design braces that may be capable of obtaining correction of the deformity. The elastic behaviour of child and adult spine differs because of both physiologic and pathologic conditions. In child scoliosis an abnormal geometry causes a persistent stress concentration in crucial areas of the motion segment. This induces a progressive elasto-plastic strain which modifies the geometry of the motion segment, thus worsening the stress concentration and the excessive strain through a vicious cycle. In adult scoliosis, on the other hand, deformation primarily affects the viscous-elastic structures, namely the intervertebral disc and the capsulo-ligamentous structures. This produces instabilty of the motion segments and slow deformation of the vertebrae through remodelling. It therefore ensues that the aim of the treatment differs in both conditions. In the child spine the aim is to prevent the motion segment deformity by means of braces which adequately modifies the stress distribution acting on the spine, thus enabling the spine to grow according to a quasi-physiological model. In adult scoliosis a stable equilibrium is required in order to prevent further deformation of the motion segment.

Adolescent↗

Corot's 'gout' and a 'gipsy' girl.

Representations of rheumatic disease in art provide insight into artistic expression, help us understand the evolution and perhaps the etiology of rheumatic diseases, and remind us of great contributions by artists in adverse circumstances. We noted hand deformities characteristic of inflammatory arthritis in Jean-Baptiste-Camille Corot's Gipsy Girl With Mandolin (1870 to 1875), National Gallery of Art, Washington, DC. Corot suffered with what probably was gout beginning in 1866. We are unaware that arthritis has been observed in Corot's subjects or that Corot's depiction of arthritis has been appreciated from the perspective of his own rheumatic disease. Examination of other Corot portraits identifies some with blurred hand details consistent with the artist's style and the remainder with normal hands. These observations suggest that the artist portrayed specific anatomic abnormalities in the "Gipsy Girl's" hand, indicating familiarity with inflammatory arthritis. It is speculative whether this was Corot's own or the model's arthritis; we favor the interpretation that Corot's gout was reflected in this particular work. We thus add a new perspective to Corot's Gipsy Girl With Mandolin-a subject with arthritis, a painter knowledgeable about arthritis, and a painting that therefore might be understood at least in part from an appreciation of the artist's specific illness.

Art↗

["Apert's foot" (in acrocephalo-syndactyly) (author's transl)].

The authors discuss foot abnormalities in Apert's syndrome (acrocephalo-syndactyly). Characteristic of these deformities are bilateral, mostly symmetrical syndactlys of the toes; incorrect hyperextension positions of the first toe with large toes in flexed positions; intermetatarsal synostoeses, above all between the first and second metatarsal bones; multiple synostoses in the tarsal region; bi- and monophalanges with pseudo-, delta- or arcuate epiphyses. A classification of Apert's foot is presented, using 68 examples from the literature and 12 cases treated by the authors. The calssification is analogous to the hand deformities in Apert's syndrome. Three types can be distinguished: In type I, toes II-IV are webbed together. However, the large and small toes are still well separated. Syndactilys of toes II-V are present in type II, and all toes have grown together in type III. The deformity can be accompanied by stress pain and difficulties in providing shoes. In this case surgical correction is indicated in childhood: the incorrect position of the large toe is eliminated. Good results can be achieved via osteotomies of the metatarsal bones and resections of the intermetatarsal bone bridges.

Abnormalities, Multiple↗

Extensive phalangeal cystic lesions: sarcoidosis limited to the hands and feet?

A young black man is presented with a 33-month course of insidious but eventually striking hand deformity and limitation of motion in the absence of any other symptoms. Radiologic and histologic findings of the digits support the diagnosis of sarcoidosis, though physical, laboratory, and chest x-ray examination show no evidence of this multisystem disease.

Adult↗

Improving functions in leprotic hands.

PURPOSE: In the developed countries there is a shift in the rehabilitation strategy from community based services to work site based services for the disabled. Leprosy disabled are more at a disadvantage that many of them have not had the benefit of disability prevention activities and harbour such hand deformities for which the only solution is community based rehabilitation. METHOD/RESULTS: Of the various modalities of rehabilitation the authors consider one for this article suitable for work site based rehabilitation as the Modulan grip-aids. CONCLUSION: Essential components of the material and its uses are described with examples.

Equipment Design↗

The long-term outcomes from a randomized controlled trial of an educational-behavioural joint protection programme for people with rheumatoid arthritis.

OBJECTIVE: To evaluate the long-term effects of joint protection on health status of people with early rheumatoid arthritis (RA). DESIGN: A four-year follow-up of a randomized, controlled, assessor-blinded trial was conducted. SETTING: Two rheumatology outpatient departments. PARTICIPANTS: People with rheumatoid arthritis less than five years since diagnosis. INTERVENTIONS: Two 8-hour interventions were originally compared: a standard arthritis education programme, including 2(1/2) hours of joint protection based on typical UK occupational therapy practice (plus 5(1/2) hours on RA, exercise, pain management, diet and foot care); and a joint protection programme, using educational-behavioural training. MAIN MEASURES: Adherence to joint protection, pain, hand pain on activity, Arthritis Impact Measurement Scales 2 and Arthritis Self-efficacy were recorded at 0 and 4 years. RESULTS: Sixty-five people attended the joint protection and 62 the standard programmes. Groups at entry were similar in age (51 years; 49 years), disease duration (21 months: 17.5 months) and use of nonsteroidal anti-inflammatory and disease-modifying drugs. At four years, the joint protection group continued to have significantly better: joint protection adherence (p=0.001); early morning stiffness (p=0.01); AIMS2 activities of daily living (ADL) scores (p=0.04) compared with the standard group. The joint protection group also had significantly fewer hand deformities: metacarpophalangeal (MCP) (p =0.02) and wrist joints (p=0.04). CONCLUSION: Attending an educational-behavioural joint protection programme significantly improves joint protection adherence and maintains functional ability long term. This approach is more effective than standard methods of training and should be more widely adopted.

Activities of Daily Living↗

Arthritis as a manifestation of self-mutilation in childhood.

Traumatic arthritis resulting from self-aggression is rarely encountered in children. Differentiation from child abuse and common causes of childhood arthritis is difficult and rests upon a high level of suspicion. We describe a 10-year-old girl with hand deformities associated with joint pain and swelling managed as juvenile rheumatoid arthritis for 3 years. Reevaluation revealed both physical and radiographic evidence of recurrent trauma. Psychiatric assessment confirmed the diagnosis of autoaggression leading to self-mutilation and psychosocial rehabilitation was essential in successful management.

Arthritis↗

[Free multiple flaps of lower extremity for severely burned hand reconstruction].

OBJECTIVE: To introduce the free multiple flaps of lower extremity based on the anterior tibital vascular pedicle for primary repair of the complex burned hand deformities. METHODS: From September 2000 to February 2003, the lateral leg flap, dosalis pedis flap and trimmed first toe based on the anterior tibial vascular pedicle were utilized to reconstruct the thumb and repair the first web, thenar, wrist or palmar scar contracture simultaneously in 6 patients. The flap size of lateral leg and dosalis pedis ranged from 4 cm x 10 cm to 7 cm x 10 cm and from 5 cm x 10 cm to 9 cm x 12 cm, respectively. RESULTS: Six cases were treated and followed up for 6 weeks to 1 year. The transplanted flaps survived with satisfactory recovery in function and appearance of the burned hand. The function of donor lower extremity was not damaged. CONCLUSION: The procedure of the free multiple flaps of lower extremity based on the anterior tibial vascular pedicle is reliable and effective for primary repair of burned hand.

Adolescent↗

Skeletal deformities of the denervated hand in Hansen's disease.

Skeletal deformities of the denervated hand are subtle in their development and pose a more formidable problem than do primary deformities of Hansen's disease. Basic osseous changes consist of concentric, longitudinal, or the longitudinal varying methods of absorption. Knowledge of their pathogenesis provides the basis for their management. Prevention of deformity and conservative management of secondary complications are necessary to salvage as much of the hand as possible.

Bone Resorption↗

New form of idiopathic osteolysis: nodulosis, arthropathy and osteolysis (NAO) syndrome.

We describe 10 patients (6 females and 4 males) from 6 unrelated families with an autosomal recessive disease characterized by simultaneous presentation of nodulosis, arthropathy and osteolysis. They were followed up regularly at King Faisal Specialist Hospital and Research Center in Saudi Arabia for clinical evaluation, serial blood work-up, and evaluating radiological changes. Nodulosis and arthropathy were the clinical criteria for inclusion in this study, and the ten patients fulfilled these criteria. All patients had nodulosis and distal arthropathy. Eight patients (80%) presented with deformed hands and four (40%) with painful hands. All patients had parents who were first cousins and three families had more than one affected child, the finding suggesting autosomal recessive inheritance. Osteopenia and undertubulation of bones distally more than proximally, and upper limbs affected more often than lower limbs, were found in all patients. Osteolysis was seen in carpal and tarsal bones. Other common findings were sclerotic cranial sutures, brachycephaly, and broad medial clavicles. This novel phenotype should be considered in the differential diagnosis of chronic arthritis. Familial arthropathies are more often seen in communities where interfamilial marriage is common. Such a collection of patients is ideal for homozygosity mapping of the disease locus.

Adult↗

Chromosomal localisation of a developmental gene in man: direct DNA analysis demonstrates that Greig cephalopolysyndactyly maps to 7p13.

Greig cephalopolysyndactyly syndrome (GCPS) is a rare autosomal dominant form of complex polydactyly. GCPS has been tentatively assigned to chromosome 7 on the basis of association of the condition with balanced translocations involving the short arm of chromosome 7 (7p13) in two families. Seven GCPS pedigrees with no chromosome abnormality were studied, and linkage was demonstrated between GCPS and the DNA sequence coding for the receptor for epidermal growth factor (localised to 7p12-13) (Z = 3.17; O = theta).

Chromosomes, Human, Pair 7↗

Skeletal dysplasia syndrome with progeroid appearance, characteristic facial and limb anomalies, multiple synostoses, and distinct skeletal changes: a variant example of the Lenz-Majewski syndrome.

Here we report a 10 year-old mentally retarded, deaf boy with a unique pattern of anomalies: progeroid appearance, characteristic facial and limb anomalies, multiple synostoses, and distinct skeletal changes. He represents a variant example of "hyperostotic dwarfism" as delineated by Lenz and Majewski.

Abnormalities, Multiple↗

Intratendon sheath corticosteroid treatment of rheumatoid arthritis-associated and idiopathic hand flexor tenosynovitis.

Flexor tenosynovitis (FT) is a common manifestation of rheumatoid arthritis (RA), contributing to hand deformity and manual dysfunction. The efficacy of intratendon sheath corticosteroids was assessed by reviewing the results of such treatment in 173 episodes of FT documented in 46 patients with definite or classic RA. Ninety-three percent of initial episodes resolved completely for 3 or more months (median: 25 months); tenosynovitis did not recur in 59%. The likelihood of a favorable response did not diminish with treatment of recurrent FT in a given digit. Comparable results were found in 52 FT episodes observed in 38 non-RA patients. No tendon sheath or soft tissue infection or tendon rupture ensued in either treatment group. The response was influenced by the specific corticosteroid preparation selected. Based on this experience, a trial of intratendon sheath corticosteroid injections prior to surgical intervention is recommended for RA patients with FT.

Arthritis, Rheumatoid↗

Clinical features of the arthritis of mixed connective tissue disease.

Seventeen of 19 patients with mixed connective tissue disease (MCTD) had arthritis as a significant initial feature of their disease; 8 were given an initial diagnosis of rheumatoid arthritis (RA) and 4 received chrysotherapy. RA-like hand deformities were present in 35% and contractures and/or persistent loss of joint motion in 47%. Joint radiographs showed abnormalities in 41% and included erosions and/or cysts in 30%. The arthritis of MCTD may be both erosive and deforming and this disease should be considered in patients presenting as RA with unusual features.

Adolescent↗

Early rheumatoid arthritis--onset, course, and outcome over 2 years.

Eighty-nine patients, 33 men and 56 women, with early definite rheumatoid arthritis were followed for 2 years. Two-thirds were seropositive. About 1/3 were eventually treated with second line drugs. The disease mostly had an insidious onset initially involving the finger joints. Early remission occurred in 16%. Patient relevant measures such as pain, patient's overall assessment of disease activity and anxiety decreased significantly. Disability evaluated by the HAQ disability index remained at a low level. The joint damage score (JDS) in hands and feet increased steadily and only 18% were nonerosive after 2 years. One-third developed hand deformities which was associated with higher JDS. A joint function index (SOFI) correlated significantly with JDS. Twenty-eight percent had a slower rate of joint damage progression the second year. There was no significant correlation between JDS and disease duration. Six patients developed rapidly progressive damage in larger joints, five in the hip joints and one in the shoulder joint, all requiring joint replacement. The ability to predict outcomes with clinical and laboratory variable obtained at entry was of limited clinical usefulness. By applying discriminant analyses 67%-80% of the cases who fared worst regarding clinical, functional, and radiological features could be correctly classified.

Adult↗