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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A bent-limb syndrome in lambs raised in total confinement was characterized by curvature of the forelimbs. Radiographic findings included flaring of the affected long bone and thinning of the growth plate. The main histologic change was endochondral dysplasia of the long bone. In feed samples, all trace minerals analyzed were within recommended concentrations except iron, which was much higher (400 ppm dry matter) than the normal requirement of lambs (70 ppm). All mineral concentrations in serum were normal except those of inorganic phosphorus and iron, which were higher. Results of soft tissue and bone mineral analyses were normal. Altering the ratio of calcium and phosphorus did not affect the incidence of disease, but intramuscular administration of massive doses of vitamin D3 and reducing the amount of dietary iron had a prophylactic effect. The increase in serum phosphorus was probably related to the dietary excess of iron, which probably decreased vitamin D metabolite formation in the kidney, which in turn could be prevented by massive doses of vitamin D3.
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PMID:Bent-limb syndrome in lambs raised in total confinement. 21 16

Eyes from cardiomyopathic hamsters (UM-X7.1 strain) were examined histologically for evidence of ocular defects. Changes observed included microphthalmia, scleral ectasia, scleral rupture, keratoconus, retinal detachment, retinal dysplasia, retinal fragmentation, retinal thinning, fibrosis of iris and ciliary body, ectopia lentis, and cataract formation. Lesions characteristic of cardiomyopathic hamsters were observed in the myocardium and skeletal muscle. This strain may be a suitable animal model to study the pathogenesis of ocular changes seen in certain congenital connective tissue disorders in man.
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PMID:Ocular abnormalities in the myopathic hamster (UM-X7.1 strain). 41 Jul 51

Pyle disease is a rare genetic skeletal disorder which is conventionally classified with craniotubular dysplasias. The radiographic manifestations in three affected adults included widening of the metaphyseal portions of the long bones which extended through a major portion of the diaphyses, with cortical thinning and mild cranial sclerosis. The femora presented the characteristic Erlenmeyer flask configuration. Pyle disease is clinically, radiographically and genetically distinct from craniometaphyseal dysplasia, a relatively common condition with which it has been confused.
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PMID:The radiological manifestations of metaphyseal dysplasia (Pyle disease). 46 17

An Afrikaner kindred in South Africa had a dominantly inherited skeletal dysplasia-blindness-deafness syndrome. The affected individuals had reduced stature and a round, flattened facies. The bone changes resembled multiple epiphyseal dysplasia with additional minor abnormalities in the phalanges, femoral heads and spine. Progressive myopia, retinal thinning and crenated cataracts led to visual disturbance, while conductive deafness represented the third component of the triad. The syndromic significance of associated asteroid hyalosis is uncertain.
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PMID:Dominant inheritance of multiple epiphyseal dysplasia, myopia and deafness. 69 54

Analysis of a further series of 125 consecutive unselected adults who were admitted to hospital with hypertension has advanced the study of arterial abnormalities and parenchymal hypoplasia, as demonstrated by selective renal arteriography, further in the direction of the parenchyma. An index of arterioparenchymal thinning is described. The authors list the features and incidence of polar arteries arising from the aorta (46%), polar arteries of non-aortic origin (31%), stenosing dysplasia (26%) and other arterial malformations, as well as biapical hypoplasia (67%), monofocal hypoplasia (37%), and the main types of renal dysgenesis (30%) which they found. The incidence of these abnormalities confirms the previous study of polar arteries arising from the aorta, and gives much more extensive information on the topic of parenchymal hypoplasia in so-called essential hypertension in the adult.
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PMID:[Kidney parenchymatous hypoplasia and arterial dysplasia in adult arterial hypertension. Data of selective renal arteriography]. 81 82

A new clinico-pathologic entity is described. It is defined as osteofibrous dysplasia of long bones, and is based on twenty two personal observations to which are added seventeen cases from the literature. This dysplasic congenital lesion is clearly differentiated from fibrous dysplasia by clinical, radiographic and histological characteristics and by its clinical course. These features may be summarised as follows: 1) Slight predominance of the male sex. 2) Very early age of onset either at birth or in the first years of life. 3) Site almost exclusively tibial, sometimes also in the fibula. Localisation predominantly in the middle third of the tibial diaphysis, but sometimes in the distal or proximal third. In the fibula, it is always at the distal third. 4) The lesion is painless and generally causes bony enlargement. There is often slight anterior bowling and more rarely, slight varus of valgus bowing. Pathological fracture may occur; rarely there is a pseudarthrosis. 5) The radiographic appearances are very characteristic, with enlargement of the bone, intracortical osteolytic lesions with thinning or disappearance of the external cortex, sclerotic reaction on the medullary aspect, and narrowing of the medullary canal. 6) The histological features are also typical, consisting of fibrous tissue enclosing bone trabeculae lined by osteoblasts and a "zonal" architectural pattern. 7) Sometimes the lesion tends to heal spontaneously in the very early years of life; in other cases it is moderatley progressive. It relapses frequently after curettage, but such recurrences are generally non-progressive. In some cases slight anterior bowing persists permanently. 8) Surgery should be restricted to patients over the age of five in whom the lesion is extensive, with imminent or actual pathological fracture, and to the rare cases of pseudarthrosis. The results are good even in cases of relapse or pseudarthrosis. The correction of residual bowing, if indicated, can safely be carried out with one or more osteotomies at the age of ten to twelve years.
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PMID:Osteofibrous dysplasia of long bones a new clinical entity. 102 9

Although the complete pathogenesis of cartilage damage in canine hip dysplasia has not been completely worked out, it appears that joint laxity with resultant excessive stresses upon articular cartilage is the initiating factor. Grossly, this damage is characterized by cartilage thinning with potential exposure of subchondral bone, osteophyte formation, and pannus formation. Microscopically, there is a marked variation in chondrocyte cellularity, with focal regions of hypocellularity and chondrocyte clusters and synovial lining cell hyperplasia and hypertrophy as well as a decrease in proteoglycan content. This latter change has been substantiated biomechanically. Ultrastructural studies indicate that there is extensive alteration of collagen organization within the cartilage.
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PMID:The pathology of canine hip dysplasia. 160 71

Epidermolysis bullosa encompasses a group of rare disorders characterized by marked skin fragility and blister formation. In patients with dystrophic epidermolysis bullosa, skeletal and soft-tissue abnormalities are an important feature. An analysis of the musculoskeletal manifestations in 19 patients is presented. In the hands and feet, features included generalized osteoporosis, wedge-shaped thinning and hooking of distal phalanges, overconstricted bones, acro-osteolysis, flexion contractures, metatarsal and metacarpal subluxation, distal trophic changes, webbing of digits, encasement of the whole extremity in a pouch of skin, soft-tissue calcification and retarded skeletal maturity. Previously undescribed findings in the hands and feet are bony ankylosis of the proximal interphalangeal joints, resorption of the metatarsal and metacarpal heads, shortened metatarsal bones, carpal and tarsal fusion and destruction, and cystic changes of the distal radius and ulna. In the remainder of the skeleton, hip dysplasia with premature osteoarthritis, knee joint bony ankylosis and thoracic and thoraco-lumbar scoliosis are other undescribed findings.
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PMID:The musculoskeletal manifestations of epidermolysis bullosa: an analysis of 19 cases with a review of the literature. 162 78

We studied six cases of unilateral hydrocephalus detected prenatally to analyze the sonographic features of the abnormality and to determine the cause and clinical outcome. In all cases, third-trimester sonograms showed marked unilateral lateral ventriculomegaly (mean atrial width, 4.4 cm) and normal contralateral lateral, third, and fourth ventricles. Five of the six cases had marked thinning of the cortical mantle on the affected side and shift of midline structures to the contralateral side. The causes of unilateral hydrocephalus were agenesis or stenosis of the foramen of Monro in three cases, transient obstruction of the foramen in one fetus with an intraventricular hematoma, underlying brain dysplasia in one fetus with a variant of holoprosencephaly, and undetermined in one case. All six neonates had placement of a ventriculoperitoneal shunt catheter; four of these have had normal cognitive development at follow-up. The remaining two infants have moderate to severe developmental impairment. Unilateral hydrocephalus is a rare anomaly that can be recognized by prenatal sonography. Even though unilateral ventriculomegaly may be marked, early diagnosis and treatment may result in a favorable clinical outcome.
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PMID:Unilateral hydrocephalus: prenatal sonographic diagnosis. 189 14

When deprived of form vision during postnatal development, tree shrews reliably develop an axial myopia characterized by elongation of the vitreous chamber, zonular dysplasia and a slight reduction in lens weight and thickness. Corneal flattening has been observed in animals visually deprived by eyelid suture but is absent in animals visually deprived with an opaque goggle. The sensitive period for myopia development starts about 15 days after the eyes open and sensitivity remains high for about 3-4 weeks thereafter. Recovery from experimental myopia can occur in tree shrews that are visually deprived using goggles for a short period. Blockade of action potentials from ganglion cells in deprived eyes by intravitreal injections of tetrodotoxin (TTX) does not prevent the development of myopia, suggesting that local retinoscleral mechanisms can contribute to experimental myopia in this species. Open eyes receiving intravitreal injections of either saline or TTX have shorter vitreous chambers than control eyes, suggesting that puncturing the globe reduces forces within the eye that contribute to its expansion. Animals treated intraperitoneally with lathyritic agents to block collagen cross-linking for three weeks during a 75-day period of monocular visual deprivation develop a very large myopia in the visually deprived eye that is accompanied by a large vitreous chamber elongation and marked thinning of the posterior sclera. The results from studies in tree shrews are consistent with the suggestion that an internally driven expansion acts in concert with ocular growth to increase the axial length of the eye, helping to move the eye from hyperopia toward emmetropia. The resistance of the sclera and/or choroid to this expansion may be affected by activity within the retina. Increased retinal activity associated with achieving a clear image on the retina may result in increased resistance to expansion, helping to hold the retina at the focal plane. Recovery may occur by a slowing of axial expansion while the optical surfaces proceed toward adult values.
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PMID:Experimental myopia in tree shrews. 208 76


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