Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Several conclusions seem evident from this study : firstly radiation myelopathies exist without any doubt; secondly clinical observation, even very attentive, during irradiation is perfectly blind regarding this subject; lastly, one can only, at this time, attempt to anticipate medullary accidents caused by irradiation. Radiation myelopathies exist incontestably. We report 27 new cases which are added to the more than 500 cases already analysed in the world litterature. The improvement of results of cancerology and in particular of radiotherapy make and will continue to make the number of observations published increase. However, if there is no doubt as to existence of these myelopathies, discussions persist concerning their nature : purely vascular, cytotoxic, probably mixed, perhaps maintained and prolonged by a superimposed immunologic phenomenon. Prevention is the sole method at our disposal to be effective. It must be applied as much to the patient as to the technique of irradiation. With regard to the patient treated in a medullary volume, several factors are probably favorable to the development of myelopathy and must cause one to modify eventually the technique of radiation proposed : the existence of anterior vertebral medullary pathology, whatever its nature; two ages demonstrate increased incidences : the young which have relative immaturity of tissue (we report 4 cases patients less than 25 years old), and the old, whose chances of accumulating associated pathologies are great, especially as systemic hypertension and arteriosclerosis are likely to have played a favorable role; the patients for whom restraint is difficult or who present disrupted regions anatomy are qually much more fragile. With regard to the technical plan, several factors incontestably favor the appearance of radiation myelopathy : large medullary volumes irradiated, especially when they encompass the zones of vascular medullary junction; the overlap of fields involving the spinal cord; the reduction of fields too close to the spinal cord not allowing at least 1 cm margin of relative security; the association of physical agents in the measure to which the global dosimetry is uncertain, i.e. in particular the use of high energy electrons for boost dosage, the intensity of which must be chosen with the greatest prudence; finally and most importantly, it seems desirable to us not to surpass at the level of the spinal cord, treating 5 times per week, a dose of 5 000 rads with fractions of 200 rads of 4 500 rads with fractions of 250 rads, and of 4 000 rads with fractions of 300 rads. Can one reasonable pretend always to foresee all radiation myelopathies? No, for on the one hand there exist authentic cases which have occured after doses which were below the limits of tolerance which we have indicated above, in accordance with others authors, and on the other hand, the necessity of sterilising certain inoperable tumors obliges one sometimes to deliver to region of the spinal cord aggressive doses.
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PMID:[Late progressive radiation myelopathies. A study of 27 cases]. 81 47

The comparison of the spiral uterine arteries structure, umbilical vessels and placenta is performed in 16 women with normal haemodynamics and in 31 women with disturbances of mother-foetus circulation, and the data obtained were compared with results of dopplerometry made before the delivery. Three clinical groups are distinguished according to the predominant localization of the resistance to the blood flow: the first--with the domination of the spiral arteries obliteration (lack of gestational changes, hyperplastic arteriosclerosis) with a characteristic ultrastructural pathology of the syncytiotrophoblast microvilli, rheological disturbances in the intervillous space and large necroses of villi; the second--with a predominance of early immaturity of the stroma and capillaries or sclerosis and compression of foetal villous capillaries, i. e. with a reduction of a capillary bed and increase of the resistance to the blood flow; the third--with a predominance of the umbilical arteries narrowing and supporting villi and signs of a blood stasis in the foetal veins. Morphologic heterogeneity of the mother--foetus circulation disturbances should be taken into consideration when choosing obstetric tactics.
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PMID:[Pathomorphology of spiral uterine arteries, umbilical vessels and placenta in disorders of maternal-fetal circulation]. 179 16

The Hutchinson-Gilford progeria syndrome (HGPS) is a very rare, but well known inherited condition of uncertain etiology in which features of premature and accelerated aging are mixed with those of delayed maturity and immaturity. Appearance at birth and birth weight are usually normal but growth typically slows after 1 year. All organ systems undergo degeneration to such an extent that the patient resembles an old man or woman. Short stature, micrognatia, alopecia, sculptured nose, prominent scalp veins, loss of subcutaneous fat, prominent joints, hyperlipidemia and early arteriosclerosis characterize the syndrome. Skeletal compromise includes hypoplasia and dysplasia, persistent open fontanelles, severe osteolysis and pathological fractures. There are no intellectual deficits in patients with this syndrome, and intelligence is unaffected. The life span in progeria is shortened by early arteriosclerosis. In this case, we review the characteristics of the severe osteolytic compromise in distal arms and limbs and bone deformities in a case of an 8-year-old girl, who was admitted to our hospital with short stature and loss of hair. On examination, the child had the major clinical criteria for HGPS as well as severe alterations in osteogenesis, including craniofacial disproportion, short and sculptured nose, delayed dentition, severe scoliosis, clavicular deformity and asymmetrical and hypoplastic arms and legs. Generalized osteopenia and severe osteolytic compromise in distal extremities were found by X-ray examination. In summary, we report the case of an 8-year-old girl who meets the diagnostic criteria for HGPS with severe involvement of her bones and joints with a review of the current literature and a possible therapeutic approach.
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PMID:Severe bone changes in a case of Hutchinson-Gilford syndrome. 1238 48