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At least 487 records · Page 27Linked to original sources
PubMed1988

The prevalence of rheumatoid arthritis in a rural African population.

We report the findings of an epidemiological study of the prevalence of rheumatoid arthritis (RA) in a rural population. The study was conducted in Venda, a very remote part of South Africa. Much of the population still follows an age old traditional lifestyle. A detailed hut-to-hut survey of 543 respondents comprising 97% of the population of one village was conducted. This included clinical, serological and radiological studies. This was supplemented by questioning local traditional doctors and showing them photographs of typical hand deformities to ascertain if they knew of any cases with these deformities. The 3 hospitals draining the area were also surveyed. The detailed survey of 543 respondents revealed no cases of definite or probable RA using a modification of the Rome criteria. No cases were recognized by the traditional doctors. The 3 hospitals together had 14 cases of definite RA out of a population of about 520,000. This gave a prevalence of 0.0026%. The marked difference in the prevalence of RA in this population as opposed to other Southern African studies as well as American and European studies is discussed.

Adolescent↗
PubMed1988

Cataract surgery on leprosy patients.

All patients who had cataract surgery at the Schieffelin Leprosy Research and Training Centre, Karigiri, India, between January 1979 and April 1985 were studied to find out the outcome of that surgery. These patients included 291 leprosy cases and 89 nonleprosy cases. Postoperative complications were slightly higher among leprosy patients compared to the nonleprosy cases. Visual recovery was marred by preoperative corneal opacities in some of the leprosy patients. Eyes with chronic insidious type of iridocyclitis did not produce any devastating results postoperatively. Patients whose skin smears were still positive for leprosy bacilli did not show any major complication. All leprosy patients should be offered the benefit of cataract surgery for restoring sight because blindness in leprosy would mean a double handicap if they are already suffering from insensitive, deformed hands and feet.

Adult↗
PubMed1978

Response of generalized granuloma annulare to alkylating agents.

The conditions of three patients who had generalized granuloma annulare responded favorably to treatment with alkylating agents. This mode of therapy in two cases was chosen on the basis of recent evidence that implicates delayed hypersensitivity mechanisms as forming part of the pathogenesis of granuloma annulare. Two patients had rapid clearing of their lesions. The response in one of these patients was observed during therapy for a concomitant plasma cell dyscrasia. The third patient had an unusual form of granuloma annulare that had evolved during a period of 28 years, with hand deformities secondary to fascial and tendon involvement and the production of contractures and lymphedema, which responded partially to therapy. The results lend further support to data implicating cellular immunity to granuloma annulare.

Aged↗
PubMed1994

[Pachydermoperiostosis. An ultrastructural study].

BACKGROUND: Pachydermoperiostosis (PDP) is a rare genetically determined disease belonging to the group of hypertrophic osteoarthropathies. Its aetiopathogenesis remains unclear. Most hypotheses favour an exogenous stimulation of fibroblasts. METHODS: A clinically typical patient with PDP was studied by electron microscopy with particular reference to the dermis and its cellular constituents. Fibroblasts from involved skin were cultured and studied in comparison with control cells. RESULTS: Remarkable modifications of the structure of the dermis were observed, encompassing irregular caliber of collagen fibres, extracellular deposits of microfibrils and of amorphous granular substance corresponding to the Alcian blue positive deposits seen by conventional histochemistry. The in vitro growth of fibroblasts was normal. CONCLUSION: Authors reviewed aetiopathogenic hypotheses. Our data suggest a genetically determined alteration of extracellular matrix production by fibroblasts as a possible explanation for the development of PDP.

Adult↗
PubMed1994

[Consequences of early-stage rheumatoid arthritis].

The consequences of early rheumatoid arthritis were studied in a series of patients treated at University Hospital, Lund, during the period 1985-1989. Mean disease duration at enrollment was 12 months. Most of the patients have now been followed prospectively for five years, during which time disease activity and pain have increased markedly. Ability to perform the activities of daily living (ADL) was well maintained, and the level of psychological distress fairly low. Radiographic changes in the hands and feet increased markedly, hand deformity being a common sign of severe disease; and only about ten per cent of the patients remained non-erosive. Another subgroup of ten per cent of the patients manifested rapidly progressive disease, resulting in destruction of larger joints, particularly hip joints, necessitating joint replacement in one or both hips. Eighteen per cent of the patients were in remission at 5-year follow-up. At 2-year follow-up, there was a high (37 per cent) prevalence of work disability, most patients who had had to stop work having done so in the first year. Physically demanding work and difficulties in performing ADL at presentation were the best predictors of subsequent work disability. The disease had a pronounced effect on life style, ability to cope with shopping, housework, leisure activities and social activities being adversely affected in more than half the patients.

Activities of Daily Living↗
PubMed1993

Hypocomplementemic urticarial vasculitis syndrome, Jaccoud's syndrome, valvulopathy: a new syndromic combination.

We describe 3 cases of hypocomplementemic urticarial vasculitis syndrome (HUVS) with Jaccoud's hands deformity and cardiac valve disease (aortic regurgitation, mitral regurgitation, mitral disease). In one case, the valve lesions required valve replacement and later a heart transplant. Valve disease and articular deformities developed 2 and 4 years, respectively, after the onset of HUVS. This as yet undescribed combination of diseases suggests a new syndrome. Pathogenesis of periarticular and cardiac lesions is unknown. The role of Clq and anti-Clq antibody is discussed.

Adult↗
PubMedDate not supplied

[Fetus in fetu: report of a single case].

A five-year-old girl had been noted, since birth to have abdominal distension. There was no history of twinning or teratoma in the family. Physical examination discovered a 15 x 10 cm mass in the abdomen. Roentgenologic examination demonstrated the presence of an axial skeleton and long bones within the mass. MRI showed a retroperitoneal mass with components of cystic, bony and fatty tissues. When the mass was surgically removed from the retroperitoneal cavity, pathologic examination disclosed a parasitic fetus measuring 15.3 x 6.3 x 6.0 cm in size and suspended by a peduncle within a capsule. The fetus was covered with skin, sebaceous materials and black hair. There were bilateral and symmetric upper extremities with a flapper-like, deformed hand. No obvious genital organ was noted. Dissection of the midline, revealed a vertebral column with spinal cord. An atrophic skull bone containing cephalized glial tissue was noted. A thoracic cavity and scapular bone were found in the thoracic cage; however, no heart was noted. Gastrointestinal tract and kidney-like organs were seen within the abdominal cavity. Microscopically, there were glial tissues within the skull bone, spinal cord and vertebra. The upper extremities revealed two long bones surrounded by myxomatous and fibrous tissue. A well developed intestine and an ovary containing follicles with nearby lympy nodes were seen. The capsule showed fibrous tissue with calcification and hairs. It was distinguished from the teratoma by the presence of a vertebral axis and by an appropriate arrangement of other organs or limbs with respect to the axis, and corresponding to the generally recognized diagnostic criteria of fetus in fetu.

Abdominal Neoplasms↗
PubMed1996

Duplication of the PMP22 gene in 17p partial trisomy patients with Charcot-Marie-Tooth type-1 neuropathy.

Autosomal dominant Charcot-Marie-Tooth type-1A neuropathy (CMT1A) is a demyelinating peripheral nerve disorder that is commonly associated with a submicroscopic tandem DNA duplication of a 1.5-Mb region of 17p11.2p12 that contains the peripheral myelin gene PMP22. Clinical features of CMT1A include progressive distal muscle atrophy and weakness, foot and hand deformities, gait abnormalities, absent reflexes, and the completely penetrant electrophysiologic phenotype of symmetric reductions in motor nerve conduction velocities (NCVs). Molecular and fluorescense in situ hybridization (FISH) analyses were performed to determine the duplication status of the PMP22 gene in four patients with rare cytogenetic duplications of 17p. Neuropathologic features of CMT1A were seen in two of these four patients, in addition to the complex phenotype asociated with 17p partial trisomy. Our findings show that the CMT1A phenotype of reduced NCV is specifically associated with PMP22 gene duplications, thus providing further support for the PMP22 gene dosage mechanism for CMT1A.

Blotting, Southern↗
PubMed2004

[The "necktie lasso": a new technique for the simultaneous treatment of Wartenberg's sign and claw deformities in the hand due to ulnar nerve palsy].

The "necktie lasso" is a new technique that allows the simultaneous active treatment, of both Wartenberg's sign and claw deformity of the fifth and the fourth digits in the hand with ulnar nerve palsy. The flexor sublimis of the fourth digit is taken by a palmar approach. It is then divided into two strips up to the proximal part of the palm; The radial strip is used as a classical "direct lasso" to treat the claw deformity of the fourth digit; The ulnar strip is wound around the base of the fifth digit by a palmar and dorsal approaches at the level of the proximal phalanx, like a necktie, being medial to its radial pedicle, dorsal and superficial to its extensor apparatus, then lateral to its ulnar pedicle; It is then recovered in the palm and sutured to itself. From September 1998 to April 2003, this technique has been used in eight patients aged between 21 and 35 years old and suffering from post traumatic low ulnar nerve palsy. It was always very effective in dealing with Wartenberg's sign: the active adduction of the fifth digit appearing at the start of flexion. The claw deformity of the fourth and fifth digits was equally actively corrected. No complications are reported in this series. With a mean follow-up of 3 years there was no recurrence of any of the deformities.

Adult↗
PubMed1988

Deforming arthropathy of the hands in systemic lupus erythematosus.

Forty-one of 858 patients with systemic lupus erythematosus (SLE) developed clinical deformity of their hands. This deformity was clinically and radiologically different from that found in 40 patients with classical or definite rheumatoid arthritis (RA), and tended to appear early in the course of disease. Characteristics of this arthropathy included nonerosive carpal collapse; exceptional erosion of the styloid processes; Z deformity of the thumb; nonerosive ulnar deviation and subluxation of MCP joints; parametacarpophalangeal joint hook formation; scant and asymmetric joint erosions; and swan neck deformity of the fingers. Most of these changes seemed to be due to involvement of the ligaments rather than to the destructive effect of synovitis. Patients with SLE with deforming arthropathy had a higher frequency of rheumatoid factor positivity, sicca symptoms and antibodies to native DNA, whereas they had lower incidence of facial rash and photosensitivity than did those without. Other manifestations did not differ. We propose that most patients with SLE with deforming arthropathy belong to a subset of SLE rather than representing the coexistence of SLE and RA.

Adolescent↗
PubMedDate not supplied

[Hand and foot deformities in a type I trichorhinophalangeal syndrome. Studies in 3 members of a family].

The trichorhinophalangeal syndrome type I is a rare, probably autosomal-dominant inherited disorder, characterized by anomalies of the cranium and face, hair, skeleton and soft parts of the hands and the feet. Typical deformities of the syndrome in hands and feet (e.g. brachydactylie, deviation and curvature of fingers and toes due to cone-shaped joints) are explained by description of the findings in three adult members of a family.

Adult↗