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PubMedDate not supplied

Bone changes in leprosy patients with disabilities/deformities (a clinico-radiological correlation).

One hundred and ten leprosy patients (96 males and 14 females, mean age 45.3 years) with disabilities/deformities were examined radiologically to evaluate bone changes and correlating them with clinical parameters. Most patients (98) had paucibacillary leprosy. The mean duration of leprosy was 7.4 years and that of deformity was 4.1 years. Ten patients presented with reaction. Seventy-five (68.2%) patients had received a full course of antileprosy treatment. The overall prevalence of bone changes was 87.3% (96 patients); specific, non-specific, osteoporotic and facial changes were seen in 44.5%, 75.5%, 38.2% and 9.1% of the patients respectively. Among the specific bone changes, primary periosteitis (28.2%) and "bone cysts" (22.7%) were the more common findings. Among the non-specific bone changes, terminal phalangeal absorption (48.2%), soft tissue changes (44.5%) and concentric absorption (32.7%) were more common. Specific bone changes showed a significant (P < 0.05) increase with lack of or incomplete antileprosy treatment. Non-specific bone changes showed significant correlation (P < 0.05) with increasing duration of disease, lack of or partial treatment and rising disability index. Osteoporotic changes showed a significant relationship with rising disability index.

Adolescent↗
PubMed2000

[Anesthetic management of a patient with Freeman-Sheldon syndrome].

We describe a case of Freeman-Sheldon syndrome that presented some problems for anesthetic management. A 2-yr-old girl required orthopedic surgery for the bilateral lower extremities. Anesthesia was induced via a mask with oxygen (2 l.min-1), nitrous oxide (4 l.min-1) and sevoflurane (approximately 5%). Tracheal intubation by direct laryngoscopy was successfully achieved. Combined caudal epidural block was, however, avoided because spina bifida occulta was suspected. Spina bifida occulta was revealed postoperatively by X-ray. For anesthetic management of a patient with Freeman-Sheldon syndrome, the spine should be evaluated preoperatively when performing epidural/spinal anesthesia.

Abnormalities, Multiple↗
PubMed2000

[Melorheostosis: a case review and review of literature].

The authors present a review of the literature and the diagnostic difficulties in a case of melorheostosis in a 6 year old boy. Melorheostosis is characterized by longitudinal foci of subcutaneous sclerosis, limitation of motion in the joints and swelling of the knee and ankle joints. Radiologically numerous foci of hyperostosis similar to wax flowing down a burning candle have been noted.

Child, Preschool↗
PubMed2001

A method for shortening instruments using the Rasch model. Validation on a hand functional measure.

BACKGROUND: Many measurement instruments, particularly measures of hand functional ability, frequently comprise a large number of items. Reduced versions of these instruments can facilitate their use. This work proposes a new method for shortening an instrument. METHODS: The method proposed was based on a scale of item difficulty calculated using the Rasch model. It was applied on a hand functional measure comprising 67 tests. The sample included 194 patients with hand lesions. The shortened instrument obtained was compared with those provided by classic methods used in the literature, with item random choice, and with shortened versions proposed by four independent experts, two rehabilitation physicians and two occupational therapists, who are clinicians familiar with the tool. All the statistical analyses were carried out on a random sub-group of two-thirds of the sample. A cross validation was then carried out on the remaining third. RESULTS: The reduction obtained had score non significantly different from that of the original instrument. In addition, the intra-class correlation coefficient and the Cronbach alpha coefficient were high. Among the different degrees of reduction investigated, the 12-item version seemed to be appropriate. Our method appeared to provide better results in terms of discriminant validity and internal validity than the choices of the four experts. The reductions produced were also better than those obtained by classic methods based on principal component analysis and multiple linear regression, as well as those obtained by random choices of items. CONCLUSION: The method presented is pertinent and useful. The reduction obtained appeared to be better than the choices of experts and the reductions provided by classic methods. The method could be used in other fields.

Accidents↗
PubMed2004

[Jaccoud arthropathy and pyrophosphate arthropathy: a rare association].

Jaccoud's arthropathy (JA) is a chronic deformity affecting hands and feet, which are voluntarily correctable by the patients. JA was usually reported in association with rheumatic fever and systemic lupus erythematosus. We describe an exceptional association between a pyrophosphate arthropathy and JA of the hands. It is a 48-year-old woman, having a 13 years history of polyarthritis involving shoulders, elbows, hips and knees, and 4 years after, a progressive unlar deviation of the fingers of the hands. Calcium pyrophosphate dihydrate crystals were identified in the synovial knee biopsy. Hands x-rays as RMI don't identify erosions and confirmed the JA.

Arthritis↗
PubMed2005

[From gene to disease; Charcot-Marie-Tooth disease or the hereditary motor and sensory neuropathies].

Charcot-Marie-Tooth disease is a clinically and genetically heterogeneous group of inherited neuropathies. The common clinical symptoms include distal muscle weakness, wasting and impaired distal sensation, more in the legs than in the arms, and reduced or absent reflexes. Moreover, foot and hand deformities are often encountered. A distinction between a primarily demyelinating or axonal neuropathy is often possible by means of nerve conduction studies. The major groups of inheritance are the autosomal dominant CMT1 (demyelinating), CMT2 (axonal) and the X-linked type (CMTX), but there are also autosomal recessive demyelinating (CMT4) and axonal (AR-CMT2) forms. The number of genes and loci is steadily increasing, with genes encoding proteins involved in myelin maintenance and axonal function, but also with genes encoding proteins, the function of which in peripheral nerve maintenance is notyet clear. Despite the increase in the number of known genes, especially for CMT2, there are many patients in whom no mutation can yet be found.

Charcot-Marie-Tooth Disease↗
PubMed2006

[Musculoskeletal examination: from neonates to adolescents].

A large number of medical consultations are related to musculoskeletal abnormalities. Less than 10 percent of patients are referred for surgery. In most cases, history taking and a mere clinical examination are sufficient to establish a diagnosis. Useful additional tests are usually limited to standard anteroposterior and lateral x-rays and laboratory analysis when an infection- or inflammation-induced abnormality is suspected. Any physician likely to deal with children should be able to conduct this clinical examination and include some of its elements given its essential role in screening (spinal deformities for instance).

Adolescent↗
PubMed1991

Radiographic manifestations of anomalies of the limbs.

Limb anomalies and their commonly associated organ malformations are increasingly recognized in fetal life because of the use of high resolution real-time sonography. In most instances plain radiography establishes the diagnosis of limb anomalies shortly after birth. In some neonates the diagnosis is tentative until full skeletal maturity is attained. When evaluation of the soft tissues and unmineralized cartilage or ossification center is a prerequisite to early definitive therapy, computed tomography and magnetic resonance imaging are the procedures of choice.

Arm↗
PubMed1990

Recent advances in restorative surgery of extremities in leprosy.

A better understanding of the pathology and mechanisms of nerve damage has changed the plan of treatment of neuritis. A concept of "Preventive Nerve Surgery" has come up during last few years. The genesis of plantar ulcers and its presentation is better understood now. The recent developments in the treatment of paralytic deformities of hand and foot have been outlined.

Foot Diseases↗
PubMed1989

[Role of orthosis in the treatment of stiffness of the hand].

The statistical analysis of results obtained with splinting treatment of finger and hand stiffness, and derived from a series of 635 motion range measurements taken on 249 hand joints, provided us with sufficient data to identify the various factors likely to have an impact on the therapeutic outcome. A mean increase of 25.8% in the range of movements is enough to justify the use of orthosis, although this figure actually varies substantially with the patient's age, the duration of the stiffness, the latter's degree and origin, the joint involved, and the duration of the orthotic treatment. An analysis of the time factor enabled us to establish the date beyond which changes for recovery are quite limited, whereby allowing to schedule surgical mobilization.

Hand Deformities↗
PubMed1988

[Hereditary Poland syndrome with megacalycosis of the right kidney].

Poland's syndrome is characterized by congenital aplasia of the sternocostal head of the pectoralis major muscle associated with ipsilateral hand deformities (most often synbrachydactylia). 20% of patients have rib cage abnormalities, some with herniation of the lung. In some cases other associated ipsilateral anomalies (hypoplasia or absence of the nipple and/or the breast, defects of the vertebrae, dextrocardia, renal aplasia of hypoplasia, undescended testes, Möbius syndrome) have been described. We describe a patient with right-sided Poland's syndrome, the first published case associated with megacalycosis of the ipsilateral kidney. It is also the first publication of a familial case with this renal anomaly, since a brother has megacalycosis of the right kidney without Poland's syndrome. The majority of cases of Poland's syndrome are sporadic. The incidence has been estimated at between 1:30,000 and 1:80,000 of live births. The great uncle of our patient had a short forearm and only rudimentary fingers. The etiology of the syndrome is unknown. Toxic effects early in fetal life or effects on primary asymmetrical blood vessels, e.g. the subclavian artery, followed by uneven growth, are discussed as possible causes of Poland's syndrome.

Abnormalities, Multiple↗
PubMed1988

Musculoskeletal manifestations of systemic lupus erythematosus.

The musculoskeletal system is involved in nearly all patients with SLE. Transient arthralgias or arthritis are common and, in some patients, there is a progression to rheumatoid-like nonerosive hand deformities (Jaccoud's syndrome). The major disabling type of joint disease in lupus is articular osteonecrosis, often induced by high-dose corticosteroids. Rare forms of musculoskeletal manifestations of lupus include spontaneous tendon rupture, crystalline arthropathies, subcutaneous calcifications, and inflammatory myopathy.

Arthritis↗