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Biomedical subjects

M Pras

Publications and source records attributed to M Pras.

190 records · Page 11Linked to original sources

Amyloid arthropathy: characterization of the amyloid protein.

An 82 year-old man was referred for joint pain and numbness of his hands. Physical examination revealed limitation of movement of the PIP's, MCP's, wrists, shoulders and knees. There was marked synovial thickening of the wrists and atrophy of the thenar muscles of both hands due to arpal tunnel syndrome. The patient was operated on both hands, the median nerves were released and a synovectomy of the wrist was performed. Two months later, a synovectomy of the right shoulder was performed. Histological examination of tissues from the wrists and shoulder demonstrated large deposits of amyloid in the synovia. Amyloid fibrils were extracted, solubilized in 6M and were fractionated on a Sepharose 6B. All three proteins that were purified from the amyloid fibrils proved to be derived from VkI light chain by their amino terminal sequences. This is the first amyloid protein to be characterized from amyloid arthropathy.

Aged↗

Absence of interferon activity during acute attacks of familial Mediterranean fever.

The pharmacokinetics of interferon, the symptoms caused by its administration, the decreased prevalence of viral diseases in FMF patients and the fact that colchicine, the drug of choice in the prevention of FMF attacks is an interferon antagonist, raised the question whether interferon may have a role in the pathogenesis of FMF attacks. An interferon activity was not detected in sera obtained at the height of FMF attacks in 8 patients, six of them under colchicine treatment. It is possible that the interferon activity has to be searched at the very beginning of FMF attacks, since at their height it already disappeared from the serum, while the symptoms of the attack are further mediated by other interferon-induced lymphokines.

Acute Disease↗

Localized eosinophilic fasciitis in a child.

An 8-yr-old girl is described who developed eosinophilic fasciitis in the left arm from the shoulder down to the tips of the fingers, after extreme effort. The clinical and histological appearance were typical and so was the benign outcome, but the age was unusual and the localized asymmetrical distribution was outstanding and has not been described before.

Child↗

Dermatomyositis in a patient with malignant melanoma.

The association between dermatomyositis and malignancy has been a controversial issue discussed in the literature. We will describe a rare case of a 34-year-old woman with a 6-year history of malignant melanoma and new onset dermatomyositis. To the best of our knowledge only 8 such cases have been previously reported in the literature.

Adult↗

Extremely active murine amyloid enhancing factor.

OBJECTIVE: To generate and characterize a highly active amyloid enhancing factor (AEF). METHODS: AEF was obtained from amyloidotic and pre-amyloidotic mice spleens that were homogenized in 50% acetone in H2O. The grade of AEF enhancing activity was studied in relation to the procedure used to generate the AEF, the amount of AEF administered, the duration of amyloid induction and the effect of solvent and denaturing agents. RESULTS: Both priming of the splenic source of the AEF with an amyloidogen and acetone processing were essential for the AEF activity. AEF in a single intravenous dose as low as 1 nanogram per mouse induced amyloidosis in mice within 2-6 days. Polyacrylamide gel electrophoresis of the AEF showed two protein bands of molecular weight (MW) 9-11 KD not present in normal spleen homogenates. Dialysis of the AEF showed that the active components can pass through a dialysis bag with an MW cutoff of 12 KD. CONCLUSIONS: These findings suggest that ours is the most active AEF currently available and that it has active constituents of low MW ( < or = 12 KD) which appear in the spleen during amyloidogenesis.

Amyloid↗

Increased prevalence of joint manifestations in patients with recurrent aphthous stomatitis (RAS)

OBJECTIVE: To characterize the systemic manifestations and joint disease in patents with recurrent aphthous stomatitis (RAS). METHODS: The presence and features of extra-oral manifestations were determined by a rheumatologist, who examined and interviewed 64 patients, referred during 1993 to the oral medicine clinic for treatment of RAS. Controls were 65 medical staff members of a military clinic associated with the hospital. RESULTS: Based on the rheumatologist's findings and published criteria, the patients were diagnosed as suffering from RAS alone (24 patients), Reiter's syndrome (8), Behçet disease (8), familial Mediterranean fever (1), or RAS with undiagnosable extra oral manifestations (23). Thirteen patients in the last group had joint disease (p < 0.01 compared to the controls), characterized by recurrent mono- or oligoarthritis/arthralgia of short duration, affecting mostly the large joints. Conjunctivitis, pustular rash, lower back pain and urethritis/cervicitis were also common in RAS patients, but only the latter was significantly more frequent in RAS patients than in controls (p < 0.02). CONCLUSION: These findings suggest that patients with RAS have an increased frequency of a palindromic type joint disease.

Adolescent↗

Quality of life of patients with familial Mediterranean fever.

OBJECTIVE: The aim of the present study was to assess the quality of life (QOL) of patients with Familial Mediterranean Fever (FMF) and to explore its possible contributing factors. METHODS: One hundred and two FMF patients were evaluated using a QOL Scale, and were compared to 124 healthy controls. The QOL scale includes 16 items, each measured on a 7-point scale (7 indicating maximal satisfaction). RESULTS: The total QOL score of FMF patients was significantly lower than that of the controls: 81.6 +/- 19.2 vs 88.0 +/- 12.8 (p < 0.01). Male and female patients reported similar QOL scores. QOL was inversely correlated with the number of FMF attacks in the last year (r = -0.302, p = 0.002), and with the number of FMF hospitalizations (r = -0.238, p = 0.017). Patients with widespread pain, sleep disturbances and headaches had significantly lower QOL scores than patients without them. CONCLUSIONS: The QOL of FMF patients was found to be impaired compared to healthy controls. Further studies are needed to determine the exact factors affecting the quality of life of FMF patients.

Adolescent↗

Long-term effects of amyloid enhancing factor: clinical and experimental implications.

OBJECTIVE: To study the long-term effects of amyloid enhancing factor (AEF). METHODS: AEF, prepared from pre-amyloidotic mouse spleens, was injected intravenously into Swiss and ICR mice. This was followed by 3 daily subcutaneous injections of AgNO3, given at increasing time intervals from the administration of AEF. The mice were sacrificed on day 6 from the first AgNO3 injection, and the mean grade of amyloid deposition in the spleens was estimated using the crush and smear technique. RESULTS: AEF was found to have a prolonged enhancing effect, retaining its activity for more than 3 months after its administration in both mouse strains. CONCLUSIONS: Experimental animals may develop amyloidosis long time after exposure to AEF. This finding may underlie the acute reoccurrence of amyloidosis sometimes observed in patients and mice long after its resolution.

Amyloidosis↗