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Biomedical subjects

P Marie

Publications and source records attributed to P Marie.

At least 37 records · Page 2Linked to original sources
PubMed1986

Lethal osteogenesis imperfecta with amniotic band lesions: collagen studies.

An infant was born with osteogenesis imperfecta (OI) and died after 7 days. In addition, there were amniotic constriction bands and amputations of several digits of the upper and lower limbs. The radiologic picture was suggestive of type III OI. Histomorphometric analysis of the bone showed a trabecular bone volume of 15.1% compared to 26.9% for age-matched controls. This was due to a decreased apposition of matrix by the osteoblasts. Because abnormal collagen synthesis has been suggested as the underlying defect in most forms of OI, collagen studies were undertaken using intact tissues. Bone and skin collagen solubilities were strikingly reduced. Shortened type I collagen molecules, representing 25% of the total type I collagen, were produced by pepsin digestion of the demineralized bone matrix. The molecular weight of the shortened collagen, was 10 kd lower than normal for both the alpha 1 and alpha 2 chains as determined by gel electrophoresis. The bone acetic acid-soluble collagen showed few shortened alpha-chains. Twenty-five percent of the acid-soluble bone collagen was cleaved into shortened molecules by a pepsin digestion. The shortened alpha 1 chain was purified by high-performance liquid chromatography (HPLC) and digested with CNBr. The analysis of the resulting fragments by HPLC and by gel electrophoresis unequivocally demonstrated that the shortened alpha 1 chain was derived from the alpha 1(I) chains and that the pepsin sensitivity extends from the amino terminal end of the chain to the alpha 1(I) CB5 peptide, approximately 120 residues inside the triple helix. These studies show a distinct structural abnormality of type I collagen in the bone matrix of this patient resulting in an increased sensitivity of the collagen to general enzymatic proteolysis. The importance of correlating clinical and biochemical information in OI is emphasized; classification and genetic counseling based only on clinical observations are inaccurate.

Amniotic Band Syndrome↗
PubMed1986

Treatment of post-menopausal osteoporosis with phosphate and intermittent calcitonin.

The progression of osteoporosis depends on an imbalance between the relative rate of bone resorption and formation leading to a decrease in bone mass. The stimulation of bone turnover, also apparently heretical, might be necessary in order to be able to make the skeletons of elderly patients become highly cellular, a prerequisite for significant restoration of bone tissue. Given the usual correlation between bone resorption and bone formation a treatment which only increases bone turnover is unlikely to increase bone mass. For this reason, it is necessary to add sequentially a drug able to block bone destruction and lead to a transient uncoupling. Forty-seven post-menopausal women with vertebral osteopenia, with one or more atraumatic spinal compression fractures were included in a double-blind randomized study in four groups. The control group (PL) (n = 9) received a placebo injection and placebo tablets. The calcitonin group (CT) (n = 15) received CT injection and placebo tablets. The phosphate group (Pi) (n = 10) received a placebo injection and phosphate tablets. The calcitonin and phosphate group (CT + Pi) (n = 13) received calcitonin injection and phosphate tablets. The duration of treatment was six months. Transiliac bone biopsies were taken before and at the end of treatment. No significant changes were noted for the (CT + Pi) group. Trabecular bone volume increased by 31% by the end of treatment. Trabecular osteoblastic surface showed a mean increase of 143% of trabecular surface. Trabecular osteoid surface showed a mean increase of 85%.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗
PubMed1984

Extended treatment of primary osteoporosis by sodium fluoride combined with 25 hydroxycholecalciferol.

Nineteen patients suffering from primary osteoporosis, all having at least one vertebral collapse, initially received 50 mg of sodium fluoride alone per day for 6-18 months. Subsequently fluoride was associated with 25-50 micrograms of 25 OH cholecalciferol (calcifediol) per day for 6-18 months in 12 of these patients and 9 were treated for 31-58 months. As control group, 9 patients were given placebo for 6-18 months. The effect of the treatment was assessed by three methods: 1) the metacarpal index (MI) determined by radiogrammetry, 2) the calcium content of the hand bone (Ca) measured by local neutron activation, 3) the iliac bone histomorphometry. MI and (Ca) did not change significantly at any time in any group. In each group there was a significant increase in trabecular bone volume, osteoid volume, osteoid surfaces and a significant decrease in mineralization fronts. On the other hand, the changes in osteoblastic surfaces, osteoclastic surfaces, number of osteoclasts/mm2 were not significant in any group. No change was observed in the placebo group. These data suggest that the increase in the trabecular volume of fluorided bone is mainly due to the increase in osteoid which itself is due to a bone mineralization defect despite the association of calcifediol. This is probably one of the reasons why (Ca) does not change significantly.

Aged↗
PubMed1984

[Prognostic value of coronary spasm threshold determined by the ergometrine test].

The prognosis of spastic angina is difficult to determine. The object of this study was to try to evaluate the prognosis of coronary spasm on the results of provocative, ergometrine testing. Out of 708 patients with angiographically normal or near-normal coronary arteries undergoing the ergometrine test for assessment of chest pain, 78 patients with positive results were retained for study. The threshold of spasm was established in every case: this was defined as the quantity of ergometrine per kilogramme body weight required to provoke spasm. The values ranged from 1 to 12.5 micrograms/kg (average 7.58 micrograms/kg +/- 3.84). The reproducibility of the ergometrine test appeared to be very satisfactory. In the short term, only 4 out of 32 tests became negative. The test remained positive in 28 cases and the mean value of the threshold of spasm did not change significantly (5.64 +/- 3.27 to 5.52 +/- 3.18 micrograms/kg). In the long term only 2 out of 18 tests became negative. The test remained positive in 16 cases and the mean value of the threshold of spasm did not change significantly (5.68 +/- 2.96 to 6.58 +/- 3.11 micrograms/kg). The ergometrine test with a reference threshold of positivity of 5 micrograms/kg is doubly useful: this threshold value helps predict a good response to calcium inhibitor drugs: the threshold of spasm was less than this value in 6 of the 41 patients whose tests became negative after diltiazem therapy, and in 12 of 14 patients in whom the test remained positive (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary Vasospasm↗
PubMed1983

Epiphysial growth after free fibular transfer with and without microvascular anastomosis. Experimental study in the dog.

The proximal fibular epiphysis was transferred in young puppies using microvascular techniques. The study demonstrated, as have previous investigators, that free epiphysial transfer without vascular anastomosis results in death of the chondrocytes of the growth plate. Histologically, the chondrocytes do not take up labelled proline, indicating diminished metabolic activity; do not take up radioactive thymidine, indicating that they are not dividing; and there is eventual disruption of the normal histological picture. In contrast, where the microvascular anastomoses re-established the blood supply to the growth plate, the epiphyses demonstrated normal histological appearance, uptake of radioactive proline and thymidine and continued to grow but at a slightly diminished rate. It is concluded that continued growth can occur after free vascularised epiphysial transfer in the dog.

Animals↗
PubMed1982

Nonosteomalacic osteopathy associated with chronic hypophosphatemia.

We studied bone histomorphometry in 19 patients with chronic hypophosphatemia related to an idiopathic renal phosphate wasting and without histological osteomalacia. Nine patients had renal lithiasis (group 1), three had radiological osteoporosis (group 2), and seven had lumbar pain (group 3). In the whole group of 19 patients, serum phosphate levels were low (24.9 +/- 2.1 mg/l), calcium in blood was normal, calcium in urine was increased, and iPTH was low. Histomorphometric data showed decreased osteoblastic surfaces with normal resorption surfaces, normal osteoid volume and calcification front. There was no correlation between serum phosphate level and histomorphometric parameters. There was no statistical difference between the data of the 3 groups of hypophosphatemic patients. We concluded that chronic hypophosphatemia in the adult doses not always lead to osteomalacia but to an unusual osteopathy characterized by an osteopenia due to an isolated decrease in bone formation. The respective importance of phosphate deficiency and of decreased iPTH level in the pathogenesis of this osteopathy is uncertain.

Adult↗
PubMed1982

Possible link between changes in plasma 24,25-dihydroxyvitamin D and healing of bone resorption in dialysis osteodystrophy.

Histomorphometric studies of bone biopsies were performed on 12 hemodialyzed patients before and after six months of treatment with 25-(OH) and 1 alpha-(OH) vitamin D3. Patients could be classified into three groups according to bone resorption: Group I with normal bone resorption; Group II with elevated initial bone resorption unresponsive to vitamin D treatment; group III with elevated initial bone resorption sensitive to vitamin D treatment. None of the patients had histological signs of osteomalacia. In Group I, plasma concentrations of 24,25-(OH)2D and the ratio of 24,25-(OH)2D to 25-(OH) D remained in the normal range throughout the study; in Group II these parameters were subnormal initially and did not increase above normal except in one case; in Group III, plasma concentrations of 24,25-(OH)2D were high before or at the beginning of vitamin D administration and normal at the time of the second biopsy and wide variations were observed in the ratio of 24,25-(OH)2D to 25-(OH)D. No difference was found between these last two groups with regard to the cumulative dose of vitamin D derivatives administered or the changes in plasma PTH, CT, calcium and phosphate. These observations suggest a specific regulation of plasma 24,25-(OH)2D concentrations in hemodialyzed patients and a possible link (independent of circulating PTH, CT, or phosphate) between this regulation and healing of bone resorption. However, no correlation was found between plasma 24,25-(OH)2D and either one of the simultaneously measured biochemical or histological parameters.

24,25-Dihydroxyvitamin D 3↗
PubMed1978

The effect of 1alpha(OH)D3 and 1alpha,25(OH)2D3 on the bone in patients with renal osteodystrophy.

Six patients with chronic renal disease and variable degrees of renal osteodystrophy were treated for three weeks with either 1alpha,25-dihydroxyvitamin D3 (1alpha25(OH)D3) or 1alpha,hydroxyvitamin D3 (1alpha(OH)D3) and both the biochemical and osseous responses measured. The most consistent changes seen were an increase in serum calcium concentration to normal, a decrease in immunoreactive parathyroid hormone toward normal, an increase in the extent of the calcification front and a decrease in the extent of fibrous dysplasia in the marrow cavity. Two important parameters which did not change significantly were serum alkaline phosphatase activity and the osteoid volume. These data, in conjunction with that from previous studies, indicate that therapy with 1alpha,25(OH)2D3 or 1alpha(OH)D3 does not heal the osteomalacia of renal osteodystrophy, but that it does suppress the secondary hyperparathyroidism, and ameliorate the osteitis fibrosa seen in patients with chronic renal disease. They raise the likelihood that additional factors, such as metabolites of vitamin D other than 1alpha,25(OH)2D3, play a role in regulating bone formation and/or mineralization.

Aged↗