Milwaukee Shoulder syndrome: eleven additional cases with involvement of the knee in seven (basic calcium phosphate crystal deposition disease).
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Biomedical subjects
Publications and source records attributed to L M Ryan.
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Conventional radiography is the standard method of objectively identifying sacroiliitis. Single views of the sacroiliac joints can be unequivocally interpreted in 70-80% of patients with low back pain. A series of views usually correctly resolves the ambiguity in the remaining 20-30% of patients (67% correct). Computed tomography will be helpful in the few patients in whom a series of views produces equivocal interpretation.
The activity of 5'nucleotidase was investigated in rabbit chondrocytes. Studies of chondrocytes in organ culture and intact cells revealed that ecto-5'nucleotidase activity varies with the age of the animal. Specific activities of 0.29, 0.62, 1.97, and 1.39 mumole/hr/10(6) cells were observed in intact cells from 3-week-, 3-month-, 6-month-, and 2-year-old animals. Specific activities of 15.5, 33.1, 80.3, and 39.5 mumole/hr/mg protein, respectively, were observed when the enzyme protein was solubilized from disrupted cells with the detergent octyl-beta-D-glucopyranoside. Membrane-bound nonspecific neutral phosphatase activity did not vary with age. Activity of 5'nucleotidase was observed on all chondrocytes using a histochemical staining technique. Kinetic properties of the enzyme from animals of different ages were investigated. No differences were noted in apparent Km value for AMP (23 microM), pH optima (7.3), substrate competition studies, and effects of MgCl2 or CaCl2. These data demonstrate a biological variation in rabbit chondrocyte 5'nucleotidase activity with age. The variation of activity represents an apparent increase in the amount of enzyme protein rather than an alteration in kinetic properties.
Enzyme replacement therapy for a severely affected 6-month-old girl with hypophosphatasia was attempted by repeated intravenous infusions of alkaline phosphatase-rich plasma, obtained by plasmapheresis, from two men with Paget bone disease. Circulating Paget AP activity was found to have a half-life (two days) similar to that reported in adults, which did not change during a five-week period of six AP infusions. Normalization of the patient's serum AP activity was followed by better control of her hypercalcemia and hypercalciuria. Sequential radiographic studies revealed arrest of worsening rickets with slight remineralization of metaphyses, although urinary excretion of the AP substrates phosphoethanolamine and inorganic pyrophosphate was unaltered by therapy. Our findings suggest that the infantile form of hypophosphatasia results from defective production of AP rather than from accelerated destruction of circulating enzyme, and that hydrolysis of AP substrates like PEA and PPi occurs primarily in tissue rather than blood. Study of additional cases of hypophosphatasia will be necessary to assess the clinical efficacy of this form of enzyme replacement therapy.
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Computed tomography (CT) and conventional radiography of the sacroiliac joint were compared in 43 patients. CT appeared to be far more sensitive and equally specific in the recognition of sacroiliitis. In a number of patients with sacroiliitis diagnosed by both techniques, CT demonstrated abnormalities that were not demonstrated by conventional radiographs. Of those patients with clinical evidence of sacroiliitis and HLA-B27 positivity, 50% had negative or equivocal radiographs compared to 19% who had negative computed tomographic images for sacroiliitis.
Calcium pyrophosphate dihydrate crystals are found most frequently in fibrocartilaginous tissue and to a lesser extent in hyaline articular cartilage. Previous investigators found that pyrophosphate (PPi) was released into the medium by immature rabbit hyaline cartilage and osteoarthritic human cartilage in organ culture but not by normal human or mature rabbit cartilage. By employing a sensitive fluorometric assay for PPi and correcting for hydrolysis of PPi during the incubations, we detected PPi release by all normal mammalian cartilage studied. PPi release per mg wet weight of lapine and canine cartilage was paralleled by uronic acid production. Meniscal fibrocartilage, the most common site of calcium pyrophosphate deposits, also elaborate PPi.
Sixty-four patients were evaluated prospectively for a reflex sympathetic dystrophy syndrome (RSDS), using quantitative clinical measurements, high-resolution roentgenography and scintigraphy. Five separate groups were identified by their clinical features, allowing us to distinguish patients with definite or incomplete forms of the RSDS as well as 16 patients with other disorders. Scintigraphy was found to be a useful diagnostic study that may also provide a method of predicting therapeutic response. Systemic corticosteroid therapy proved to be a highly effective mode of treatment for up to 90 percent of the patients with the RSDS.
Sixty-four consecutive patients were studied for possible reflex sympathetic dystrophy syndrome (RSDS). They were divided into five groups, based upon specific clinical criteria, and the radiographic and scintigraphic findings in each group were examined. Osteoporosis was the most common radiographic abnormality, present in 69% of subjects with definite, probable, or possible RSDS, as compared with 21% opf those with RSDS. Scintigraphic abnormalities were noted in 60% of RSDS patients but in only 7% of the others. These findings included increased blood flow and enhanced periarticular radionuclide activity in the affected extremity. Of 11 patients with serial scintigraphy, six (55%) demonstrated a return to normal, symmetrical patterns following successful therapy. The scan may reflect an active, potentially reversible disorder of local blood flow in RSDS. Furthermore, the scintigraphic patterns may be useful in the diagnosis and in predicting which pattients are likely to respond to systemic steroid therapy.
Rice bodies and synovia obtained from knee joints of rheumatoid arthritis patients were solubilized by limited pepsin treatment. The quantity of each type of collagen in both tissues was determined by differential salt precipitation, cyanogen bromide peptide analysis, and SDS polyacrylamide gel electrophoresis. Rice bodies and synovial membrane contained equal proportions of Type I and III collagens with trace amounts of Type "A-B" collagen.
Collagen fibers in synovial fluid sediment were described a decade ago. Since then, tissue-specific collagen molecules (types) have been characterized. Techniques were devised to identify the collagen types in joint fluid sediment. Collagens were found in 12 of 17 pellets prepared from fluid aspirates from 17 knee joints of patients with various forms of arthritis. Collagen types I and III and polypeptide chains A and B (basement membrane collagen) were specifically identified in four of seven fluids from patients with active systemic lupus erythematosus (SLE) and in a single fluid from a patient with severe septic arthritis. This "collagen profile" was identical to that of rheumatoid synovium. Type II collagen, characteristic of hyaline articular cartilage, was found in two of six fluids from osteoarthritic joints. The presence of sufficient collagen (about 5 micrograms) to permit typing was correlated with roentgenographic evidence of joint space narrowing; the presence of the "synovial" collagen profile was correlated with decreased joint fluid pH.
The nuclear magnetic resonance linewidth of 1H in water of frog muscle was studied as a function of magnetic field strength and angle of orientation. The results suggest that the observed spectra are dominated by demagnetization field anisotropy and dispersion, but a small static dipolar interaction of the order of a few hertz man be present. Data from line-narrowing, multiple-pulse experiments also indicate the presence of a small dipolar broadening.
The methodologic variables of the UDPG pyrophosphorylase method for analysis of inorganic pyrophosphate (PPi) levels in biologic fluids are described. Use of a tourniquet in collection of blood specimens elevated plasma PPi levels from 35% to 55% above control values and may explain the differences in published normal values. The sodium pyrophosphate decahydrate used to prepare the standard solution lost 8 waters of hydration after dessication, which could result in the calculation of spuriously elevated PPi levels. Normal plasma PPi concentration was 2.18 muM with a range (95% confidence limits) of 0.58-3.78 muM. Comparison of plasma PPi in normal subjects, patients with primary osteoarthritis, and patients with calcium pyrophosphate dihydrate deposition disease revealed no significant intergroup differences.
Plasma inorganic pyrophosphate (PPi) levels rose predictably (28%) after vigorous systemic exercise and returned to baseline values after 30 minutes of rest. Plasma PPi levels were greater (36%) in femoral venous blood than in femoral arterial blood. Forearm muscular exercise, however, did not result in a detectable rise in plasma PPi levels in antecubital veins. The physiologic reasons for both the systemic exercise effect and the arteriovenous differences remain unknown. Measurement of plasma PPi for 10 consecutive days in a single normal subject under basal conditions, where these and other previously identified biologic variables (fasting and diurnal variation) were controlled, showed a coefficient of variation of 12.7%. Thus biologic variability contributed less to observed variation in plasma PPi than did methodologic errors (coefficient of variation of method = 8%). The striking reproducibility of plasma PPi in this subject suggests that this important metabolite is under rather tight homeostatic control.
Two patients with linear scleroderma (en coup de sabre) developed systemic lupus erythematosus (SLE). This association has been well documented in only one previous case. The presence of high titer antibodies to ribonucleoprotein (RNP) initially led to the diagnosis of the mixed connective tissue disease. Development of more serious clinical involvement and antibodies to Sm (case 1) or native deoxyribonucleic acid (nDNA) (case 2) helped establish a diagnosis of SLE. Use of these studies in the differential diagnosis of systemic rheumatic diseases is disucssed briefly. The presence of anti-RNAP antibodies in patients with localized scleroderma may herald a more serious rheumatic disease.