Luke's Tuberous Sclerosis Page
Skeletal Research |
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SKELETAL INVOLVEMENT IN TUBEROUS SCLEROSIS. A. K.Morozov, S.V.Lapkina, P.A.Tenijn*, M.J.Dorofeeva*, V.J.Bosin**, V.V.Kondrina**, P.V.Novikov***. Central Institute of Traumatology and Ortopedia, Priorova str.10. 2125299 Moscow, Russia; Institute ofPaediatrics & Paediatric Surgery, *Dept. of Neurology and Epileptology, ** Dept. of Functional Methods of Investigation, *** Dept. of Clinical Genetics Taldomskaya str. 2, 127412 Moscow, Russia. Skeletal involvement in pathological process in TS patients noted in45-66%. X-ray method discovers sclerosis of skeleton tissue in a variety of locations, destruction may be seen in certain flat bones, cyst like lesions in hands and feet, metatarsal periosteal new bones, and rarely osteolytic lesions (A-Hoffman, 1991).Nevertheless these works are descriptive and does not reflect the full-scale of changes in skeleton system. The aim of this work is the study of peculiarities of the skeletal pathology in TS patients on the basis of complex study of the patients by X-ray method and by X-ray bone densitometry. X-ray study included research tubular bones with hands and feet. X-ray densitometry was conducted by "Lunar DPX-L'. Standard program of investigation for lumbar vertebrae (Ll-L4) of the spine and for both femoral necks was used. Analysis of the data obtained was made automatically. The results had been compared with the population standard. To analyze the nature of skeletal pathology we had studied 15 TS patients-girls (6-14 years old), g boys (5-15 years old) as well as 3 mothers suffering with the me disease (age 34 to 41). Biochemical blood test discovered lower levels of calcium in all patients. T he X-ray of long tubular bones revealed osteoporosis lesions in 11 patients and lagging behind the bone age in 6 children. The densitometry had proved the following drop in bone density: 94% of patient shad decrease of bone density in femoral neck (479/0 of patients by 10%; 47%of patients by 20%). 60% of patients had decrease of bone density in lumbar spine (40% of patients by 10%, 13% of patients by 20% and 7% of patients by 30%). Thus the trend for lowing bone density in femoral necks is overwhelming the trend for lowing bone density in lumbar spine. The reasons for changes in skeleton structure and their interrelation with pathogenesis and the development of the disease are not clear enough. They can be regarded as the consequences of disturbances in hormonal regulation and disfunction of different glands of internal secretion as well as the consequence of long term anticonvulsant treatment. So taking into consideration the data obtained in the research. the therapy by TS patients should include medications aimed to correct the mineral metabolism. This home page is intended to be a family resource for families affected by Tuberous Sclerosis. It does not intend to constitute medical advise. Viewers are warned not to take any action with regard to medical treatment relying on the information provided on this page without first consulting the patient's physician.
Deanna Runyan-Wall E-mail address: deannadawn@lukets.org Last updated: April 5, 2008 Created: December 5, 1996 |
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