Osteogenesis imperfecta: Effect of magnesium administration on pyrophosphate metabolism

Solomons, Clive C.; Styner, J.

doi:10.1007/bf02058674

Cited by 58 publications

(23 citation statements)

References 14 publications

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“…The practical importance of these observations is that blood or other fluids and tissues to be analyzed for PPi must be obtained with minimal trauma and platelets effectively separated before analysis -or falsely high values may result. The high values for serum PPi reported by Solomons and Styner for example, probably reflect platelet PPi, at least in part (22). Whether platelet PPi has a significant physiological role as do ADP and ATP, remains to be determined.…”

Section: Inorganic Pyrophosphate In Human Plateletsmentioning

confidence: 93%

Identification of Inorganic Pyrophosphate in Human Platelets and Its Release on Stimulation with Thrombin

Silcox¹,

Jacobelli²,

McCarty³

1973

J. Clin. Invest.

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A B S T R A C T Serum inorganic pyrophosphate (PPi) levels were consistently two-to threefold higher than plasma PPi prepared from the same blood. PPi was found in platelets in amounts ranging from 1.4 to 3 nmol/108 cells, using three different techniques for quantification. These levels are approximately 800 times higher than the mean PPi concentration in normal plasma and approximate the levels of ADP found in platelets by other workers.About 50% of platelet PPi was specifically released extracellularly after stimulation with thrombin. Timed release experiments showed a pattern of release that resembled that described for ADP and ATP. This pattern was clearly different from that shown by platelet calcium, serotonin, or P-glucuronidase. Platelet inorganic pyrophosphatase was not released into the supernate in detectable amounts.

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Section: Inorganic Pyrophosphate In Human Plateletsmentioning

confidence: 93%

Identification of Inorganic Pyrophosphate in Human Platelets and Its Release on Stimulation with Thrombin

Silcox¹,

Jacobelli²,

McCarty³

1973

J. Clin. Invest.

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“…Reported abnormalities of pyrophosphate metabolism [49], abnormal leukocyte oxidative metabolism [22], and alterations of metabolic rates with increased serum thyroxine levels [13] have been interpreted as evidence of a generalized derangement of cellular oxidative metabolism in osteogenesis imperfecta. After puberty, metabolic rates in the patients studied were found to normalize [13].…”

Section: Discussionmentioning

confidence: 99%

“…After puberty, metabolic rates in the patients studied were found to normalize [13]. Magnesium oxide, which was found to reverse the pyrophosphate alterations, has been proposed [49] as a form of treatment for osteogenesis imperfecta. Previous work [1] has shown that, in hyperthyroidism, both bone formation and bone resorption are increased.…”

Section: Discussionmentioning

confidence: 99%

“…Bleckmann et al [8] found normal proline and hydroxyproline content in bone with an increase in hydroxylysine; these authors postulated a deficiency in cross-linking of collagen. Investigations of cellular oxidative metabolism have suggested that osteogenesis imperfecta is a generalized metabolic disorder with a disturbance of high energy phosphate metabolism which results in abnormal pyrophosphate metabolism [22,49].…”

Section: Introductionmentioning

confidence: 99%

“…As a result of the confusion regarding the basic abnormality in osteogenesis imperfecta, a variety of systemic treatments have been suggested for this disease, including ascorbic acid [59], sodium fluoride [2,3,31], and magnesium oxide [49]. Evaluation of the efficacy of treatment from these reports is difficult because the numbers of patients treated are small and the results are generally expressed in terms of a decrease in the number of fractures occurring in a relatively short period.…”

Section: Introductionmentioning

confidence: 99%

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Osteogenesis Imperfecta: Morphologic and Biochemical Studies of Connective Tissue

1973

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ExtractIn iliac crest biopsies from 11 patients (ages from 4 to 22 years) with osteogenesis imperfecta, cortical thickness, osteoid width, and number of osteons were less than normal at all ages. Bone surface activity (formation and resorption), measured by quantitative microradiography, was lower than normal in younger patients; the normal pubertal decrease in surface activity was not evident in patients with osteogenesis imperfecta and, consequently, bone surface activity in the older patients was higher than in age-matched normal subjects. The ultrastructure of osteocytes, bone collagen, and scleral collagen did not differ from normal; corneal collagen fibers, however, were approximately half the diameter of normal. Total urinary hydroxyproline excretion, measured in 11 patients, was below normal in all except 2 infants (less than 12 months old). Serum parathyroid hormone levels, measured in nine patients, were normal. Long term treatment with sodium fluoride (two patients) and. treatment with magnesium oxide (three patients) did not alter bone morphology or bone surface activity. SpeculationThe supposition that in osteogenesis imperfecta the connective tissue is characterized by an immature form of collagen was not substantiated by this study. The abnormal fibrillar pattern and paucity of collagen in various connective tissues suggest a qualitative defect, related to collagen maturation, as well as a quantitative deficit in the amount of collagen produced.

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Possible Predictive Tests for Malignant Hyperthermia During Anesthesia

Aldrete¹

1971

JAMA

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Osteogenesis imperfecta: Effect of magnesium administration on pyrophosphate metabolism

Cited by 58 publications

References 14 publications

Identification of Inorganic Pyrophosphate in Human Platelets and Its Release on Stimulation with Thrombin

Identification of Inorganic Pyrophosphate in Human Platelets and Its Release on Stimulation with Thrombin

Osteogenesis Imperfecta: Morphologic and Biochemical Studies of Connective Tissue

Possible Predictive Tests for Malignant Hyperthermia During Anesthesia

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