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PubMed1987

Postoperative course of patients treated with iliac osteocutaneous free flaps. A two- to five-year follow-up study.

The clinical and radiologic course was reviewed in seven patients treated with an iliac osteocutaneous free flap (iliac flap) for treatment of large bone defects in the tibia and first metacarpal bone and overlying skin. Application of the iliac flap was followed by free bone grafting in four patients and aesthetic surgery in four patients. All patients obtained solid bony union and good skin coverage; none had complaints upon walking. An iliac flap was useful as a salvage procedure for the treatment of massive defect of bone and skin in extremities, especially in the lower one-third of the lower leg. Transient osteoporosis occurred before osteosclerosis and bony union. Bone remodeling with change in the trabecular pattern preceded transformation from cancellous to cortical bone.

Adolescent↗
PubMed1987

[A case of pycnodysostosis--observation of the skull by CT scan].

A 13-year-old boy was presented to the Department of Neurosurgery, Saiseikai Fukuoka General Hospital for further examinations concerning abnormal findings in the skull radiogram taken when he struck his head. His physical features showed some characteristics the same as those of pycnodysostosis as follows--proportionate dwarfism, prominent forehead, short spoon-shaped fingers, bilateral exophthalmos. A skull radiogram revealed widely open cranial sutures with no healing of the fracture and craniotomy which was performed for an acute epidural hematoma 6 years ago. Furthermore, the mandible was hypoplastic with a virtual loss of mandibular angle. CT of the soft tissues showed somewhat dilated cortical sulci and ventricles without any structural abnormalities in the brain. CT of bone algorithm revealed specific characteristics of this disease. The paranasal sinuses were quite hypoplastic. Especially in the maxillary sinuses, frontal sinuses and mastoid air cells, none of developments of sinuses were noted, even though the middle and internal ear seemed to be normal. Moreover, the ethmoid and sphenoid sinuses were noted, although their developments were poor. The appearance of skull base was normal, including the inlets and outlets of cranial nerves or vessels and synchondroses. However, the density of the skull base, especially in the diploe, was higher than normal in Hansfield number. Furthermore, detailed measurements of skull base demonstrated that the skull base itself was also dwarfism. Pycnodysostosis is a generalized skeletal disease whose cardinal features are moderate generalized osteosclerosis and dwarfism. However, the detailed observation on the cranium by CT has not been reported. In our study, the development of sinuses in bones with intramembranous ossification are worse than that with endochondral ossification.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1986

[Changes in the femur of fur-bearing animals during hypodynamia].

Under conditions of hypodynamia the form and internal architectonics of the femoral bone undergo certain changes. In young animals these changes are of quantitative character without any signs of a manifested destruction. Later on, certain indications of focal resorption appear in the spongy substance, as does partial destruction of the beams. Gradually, definite signs of osteoporosis and osteosclerosis as a result of dystrophic alterations in the osseous tissue develop. These phenomena, though progress with age, nevertheless, develop against the background of noticeable compensatory shifts in the osseous structures: increment in the mass of some osseous beams, appearance of new strengthening osseous plates in the spongiosa. The symptom complex at adaptational rearrangement of the bone under hypodynamia should be taken into account at differential diagnosis of these conditions both in the clinic and at experimental modelling.

Adaptation, Physiological↗
PubMed1985

Autoimmunity to native type II collagen--a distinct genetic subset of rheumatoid arthritis.

HLA phenotypes were determined in 60 Caucasoid patients with rheumatoid arthritis (RA) and normal serum antibody levels to native type II collagen. Antigen frequencies were compared with 52 patients with RA who had elevated antibody levels to native type II collagen. Both RA groups were compared with 163 healthy controls. A clinical comparison of both RA groups yielded few differences, except a decreased incidence of rheumatoid factor and a positive family history of RA and radiologically, increased osteosclerosis in the RA group with elevated antibodies to native type II collagen. HLA-DR4 was increased and HLA-DR7 was decreased in the RA group with normal antibody levels to native type II collagen. A comparison of both RA groups showed an increased incidence of HLA-DR4 in the normal antibody group, whereas HLA-DR7 was increased in the elevated antibody group. In the elevated antibody group the majority of patients possessed either HLA-DR3 or DR7 both of which are in strong linkage disequilibrium with HLA-DQw2. This immunogenetic data suggests that RA patients with autoimmunity to native II collagen form a distinct genetic subset of RA.

Adolescent↗
PubMed1986

Familial nonspherocytic hemolytic anemia in poodles.

Nonspherocytic hemolytic anemia, characterized by marked reticulocytosis, hepatosplenomegaly, hemosiderosis of reticuloendothelial organs and bone marrow myelofibrosis, and osteosclerosis, was diagnosed in 5 related Poodles. The unremitting anemia was clinically evident by 1 year of age, and was fatal as early as 3 years of age. Despite intense diagnostic endeavors including RBC fragility studies, RBC enzyme assays, and hemoglobin electrophoresis, the cause of this nonspherocytic hemolytic anemia remains to be determined.

Anemia, Hemolytic, Congenital↗
PubMed1979

Industrial fluoride pollution. Chronic fluoride poisoning in Cornwall Island cattle.

An aluminum plant on the south bank of the St. Lawrence river, southwest of Cornwall Island, Ontario, Canada, has emitted 0.816 metric tons of fluoride daily since 1973; considerably higher amounts were emitted from 1959 to 1973. The plant has been designated as the "major source of fluoride emissions impacting on Cornwall Island." Chronic fluoride poisoning in Cornwall island cattle was manifested clinically by stunted growth and dental fluorosis to a degree of severe interference with drinking and mastication. Cows died at or were slaughtered after the third pregnancy. The deterioration of cows did not allow further pregnancies. Fluoride concentrations in ash of biopsied coccygeal vertebrae increased significantly with age and were dependent on distance from and direction to the aluminum plant. Fluoride in bone ash of a 7-month old-fetus exceeded 500 ppm; fluoride thus was passed transplacentally. Analyses of fluoride in ash of bones obtained at necropsy of cattle from 4 months of age to 4 to 5 years of age showed increased amounts with age. Cancellous bone retained far higher amounts than cortical bone, a reflection of the normally higher metabolic rate of cancellous bone. Concentrations exceeding 10,000 ppm fluoride were recorded in cancellous bone of a 4-to 5-year-old cow. The target cells for fluoride in chronic fluorosis were shown to be the ameloblasts, the dental pulp cells and the odontoblasts and, in bone, primarily the resorbing osteocytes and also the osteoblasts. Atrophy and necrosis of the ameloblasts were responsible for enamel defects. The existing enamel showed brown discoloration from fluoride deposits. The pulp cells underwent fibrous and osseous metaplasia and necrosis of the ectopic bone occurred. The odontoblasts were atrophic and the dentin showed brown discoloration. The resorbing osteocytes were inactive and osteosclerosis resulted. This was especially pronounced in areas of normally great apposition, i.e. in the metaphyses. The epiphyseal plate became squeezed between petrotic bone and growth was stunted. Resorption of alveolar bone surrounding the deciduous teeth was severely retarded or arrested. A delay in eruption of permanent teeth occurred; it was up to 3.5 years in incisor teeth. Interference with the resorbing osteocytes in fluorotic bone was also demonstrated by loss of collagen birefringency in such bone. Failure of bone resorption also caused retention of trabecular bone in the cortices; this was observed even in a 4-t0-5-year-old cow. In areas where modeling into osteonic bone had begun, fluoride deposits were extremely heavy but this bone showed numerous soft osteons in microradiographs. The toxic effect of fluoride on osteocytes also resulted in the death of the cells. Such osteonecrosis occurred mainly in gnathic bone. There was atrophy of the osteoblasts. Osteopenia thus resulted from osteonecrosis and osteoporosis. Subperiosteal exostoses were not observed in long bones. The degree of fluorosis in Cornwall Island cattle was severe...

Air Pollutants↗
PubMed1979

Age-related degenerative changes in the mouse mandibular joint.

The mandibular joints of ageing male ICR mice were studied by light and electron microscopy. A high incidence of degenerative joint disease was found relatively early in adult life, osteoarthrosis being evident by the seventh month. Initially the pathological changes were those of chondromalacia confined to isolated foci on the articular surface of the condylar cartilage. Later, characteristic signs of osteoarthrosis were noted, namely fibrillation, fibrous ankylosis, lipping and subchondral osteosclerosis. Ultrastructural examination indicated a marked heterogeneity in the articular lesions. Deep fissures and extensive cracks spread to the underlying matrix, and these were associated with severe alterations in its macromolecular structure. It is proposed that ageing chondrocytes produce a matrix which is increasingly incapable of withstanding normal mechanical forces, and this leads to the morphological changes recognized first as chondromalacia and then as osteoarthrosis.

Aging↗
PubMedDate not supplied

Secondary hyperparathyroidism in chronic renal failure.

The metabolic bone disease associated with chronic renal failure has been described collectively by the terms "renal osteodystrophy" or "renal-glomerular-osteodystrophy" and consists of osteomalacia, osteitis fibrosa, and osteosclerosis. The skeletal abnormalities may occur either alone or in combination with one another. An increased concentration of circulating immunoreactive-parathyroid hormone (i-PTH) is a recognized feature of patients with chronic renal failure, and the values are usually much higher than those found in patients with primary hyperparathyroidism associated with a parathyroid adenoma. It must, however, be recognized that the high circulatory concentrations of parathyroid hormone found in patients with chronic renal failure are of immunoassayable material which may or may not be of biological significance in respect of activity. A disturbance in the homeostatic control mechanism governing parathyroid hormone, the secretion rate, its metabolism, and target organ resistance to its action are of major importance in the pathogenesis of some aspects of the metabolic bone disease in patients with chronic renal failure. The pathogenesis of the secondary hyperparathyroidism of chronic renal failure, however, also involves disturbances in cholecalciferol metabolism, phosphate retention, and the uremic state per se.

Calcium↗
PubMedDate not supplied

[Osteodystrophy in children with chronic renal insufficiency in dialysis therapy].

One of the most important complication of patients with chronic renal failure is osteodystrophy. This causes skeletal deformities, growth failure, bone pain and decreased physical activity. Osteodystrophy is more frequent among children than uraemic adults. In fact, 50-80% of children with chronic renal failure may occur in metabolic bone disease and the incidence tends to be higher in those children who have been in uraemic state for a long time before starting chronic haemodialysis. Osteodystrophy is a result of: 1) lesions of rickets; 2) lesions of osteitis fibrosa: 3) osteosclerosis. In contrast to adult, metastatic calcifications are virtually never observed in uraemic children. Hyperphosphoraemia, that is secondary to the reduction of G.F.R., may be the principal responsible of hyperparathyroidism that is the main cause of osteodystrophy. Hyperparathyroidism is also maintained and increased by deficit of 1,25(OH)2D3 which is responsible for lesions of rickets. Haemodialysis may markedly improve osteitis fibrosa and it is efficacious in reversing the mineral defect. Dialysate calcium concentration should be maintained at approximately 3,5 mEq/l. In this case we can raise serum calcium. On the contrary dialysate has to be lacking in phosphorus to correct hyperphosphoraemia. It must be noted that we have to prepare a dialysate with deionized water lacking in aluminum to avoid encephalopathy compliance.

Aluminum Hydroxide↗
PubMed1984

[A case of osteopathia striata].

We report a case of osteopathia striata, which is an osseous dysplasia with linear striations of the metaphyses of long bones. Our case is very unusual as there are no associated abnormalities of the bones. Osteopathia striata should not be mistaken for osteopoikilosis. Osteopathia striata is not only a peculiar roentgenologic feature: when it is diagnosed, other abnormalities should be looked for, especially cranial osteosclerosis, which may result in complications.

Adult↗
PubMedDate not supplied

[Pustular arthro-osteitis. A new entity among rheumatic diseases?].

A patient is shown with symptoms of the anterior thoracic wall syndrome and pustulous changes on the palms an soles. In the clinical picture, a painful swelling of the medial third of the right clavicle dominated, while the general state of the patient was good. Laboratory findings showed positive tests of inflammatory activity, while the rheumatoid factors and the HLA B 27 antigen were negative. Radiologically wide-spread destructive changes of the medial part of the right clavicle were found, with signs of periostosis in the active phase of the disease and osteosclerosis in remission. The symptoms of the anterior thoracic wall have often relapsed, while the plamo-plantar pustulosis was present only at the onset of the disease. The function of locomotor system remained undamaged even after eight years of the disease duration. The disease was, after its clinical, laboratory and radiological aspect, different from all up to now known rheumatic diseases as well as from other diseases with symptoms on the locomotor system. It fits into the syndrome which was described by japanese authors under the name of "pustulous arthroosteitis".

Adult↗
PubMed1983

Pathologic fracture in systemic mastocytosis. Radiographic spectrum and review of the literature.

Systemic mastocytosis is a multiorgan disease that most commonly affects the skin and skeletal system. Radiographically, the skeletal changes in the majority of patients consist of either a wide-spread mixture of bone lysis and osteosclerosis or generalized osteoporosis. The osteoporotic form is less well known but may lead to severe generalized demineralization and pathologic fractures. Mast cells secrete a number of substances, two of which (heparin and prostaglandins) are believed to have a role in the induction of osteoporosis. Sclerotic lesions are induced by another mast cell by-product, histamine. One hundred seventy-eight cases of bony mastocytosis have been reported in the literature, including the four patients in the present report. Special staining procedures are necessary for identification of mast cells. Diagnosis may be delayed in patients who do not have the skin lesions (urticaria pigmentosa) and in the osteoporotic form of the disease.

Adult↗
PubMed1984

Progressive diaphyseal dysplasia: genetics and clinical and radiologic manifestations.

Progressive diaphyseal dysplasia was found in a three-generation family including 13 affected individuals, the largest family reported to date. Our study confirms that progressive diaphyseal dysplasia, also known as Engelmann's or Camurati-Engelmann disease, is an autosomal dominant disorder with variable osseous and muscular manifestations. Disease distribution among patients, within a given patient, or even in individual bones is unpredictable. The femur is the most commonly and severely affected bone and hence most useful for radiographic screening of possible patients. Radiographs provide a meaningful assessment of disease activity and extent. The severity of symptoms is generally proportionate to severity of involvement shown by roentgenography. Exophthalmos due to osteosclerotic dysplasia of the skull occurred in more than half of the patients with progressive diaphyseal dysplasia. Twelve-year follow-up of this family, with affected individuals ranging in age from 6 months to 12 years, indicates that progressive diaphyseal dysplasia may progress or become quiescent and be remarkably inactive despite advanced osteosclerosis and structural deformity.

Adolescent↗
PubMed1984

Double balanced de novo translocations involving chromosomes 4/15 and 5/12 in a mentally retarded boy.

This report describes a compound structural rearrangement containing two apparently unrelated reciprocal translocations, one between chromosomes 4 and 15, the other between 5 and 12. It is suggested that the patient's mental retardation and osteosclerosis may be the result of the loss of a small amount of chromosomal material and/or position effect accompanying these translocations.

Adolescent↗
PubMedDate not supplied

[The hand of the patient under periodic hemodialysis treatment. Comparative radiographic and xeroradiographic aspects].

Renal osteodystrophy is the skeletal response to long-standing chronic renal disease. The radiographic features include hyperparathyroidism (osteitis fibrosa cystica), rickets or osteomalacia, osteoporosis, osteosclerosis and soft tissue and vascular calcifications. Many investigators have verified that changes in the phalanges of the hand are the most sensitive indicator of renal osteodystrophy, especially subperiosteal bone resorption. The hands of 50 chronic dialysis patients were evaluated for at least one year for tuftal resorption (acro-osteolysis), subperiosteal and intracortical bone resorption, and periosteal new bone formation (periosteal neostosis), using fine detail radiography and optical magnification. At last control, only in a group of 17 subjects (34%), xeroradiography of the hand was also performed. Results indicate the importance of the use of fine detail radiography in the early diagnosis of renal osteodystrophy and that serial radiography of the hand is a proper method of evaluating progressive bone resorption. In addition, a significant difference (p less than 0.05) is demonstrated between xeroradiography and radiography to evaluate periosteal neostosis.

Adult↗
PubMed1983

Effect of dietary calcium on the response of bone to 1,25 (OH)2D3.

The purpose of this investigation was to evaluate, by static and dynamic histomorphometry, the comparative influence of dietary calcium on the effect of 1,25(OH)2D3 on bone. Young adult female rats were fed a diet containing 0.3% phosphorus and 0.05%, 0.5%, or 1.0% calcium. Rats in each group received placebo, or 27 ng or 135 ng of 1,25(OH)2D3, intraperitoneally, for 10 days. A dramatic osteosclerosis and hyperosteoidosis characterized by an increase in metaphyseal hard tissue percentage and percentage metaphyseal osteoid, a decrease in osteoclasts per millimeter of trabecular surface perimeter and longitudinal growth, and hypertrophy of osteoblasts developed in the proximal tibial metaphysis of rats fed 1.0% calcium diet and given 135 ng of 1,25(OH)2D3 compared with placebo-treated rats fed the same diets. Similar histomorphometric changes, with the exception of increased metaphyseal hard tissue percentage, occurred in rats fed 0.5% dietary calcium and treated with 135 ng of 1,25(OH)2D3. Metaphyseal hard tissue percentage, percentage metaphyseal osteoid, and osteoclasts per millimeter of trabecular surface perimeter were not affected by treatment with 135 ng of 1,25(OH)2D3 in rats fed a 0.05% calcium diet. Serum calcium was significantly elevated in all 1,25(OH)2D3-treated rats. Dietary calcium appears to modulate the effects of 1,25(OH)2D3 on bone. Only diets containing adequate dietary calcium permit exogenous pharmacologic levels of 1,25(OH)2D3 to produce a positive skeletal balance either by decreasing osteoclasis and/or increasing bone matrix synthesis.

Animals↗
PubMed1984

Erdheim-Chester disease. A case report.

In a 57-year-old woman dizziness, staggering, altered mental status, and bilateral pain and cramping in the lower extremities developed. Extensive investigation yielded evidence of renal and long-bone lesions, with the latter being primarily metaphyseal and diaphyseal. Needle biopsy of the tibial marrow revealed the presence of lipid-laden histiocytes, osteosclerosis, and osteoclasts. A diagnosis of Erdheim-Chester disease was made; this is the 16th known case of the disease. The patient responded well to steroid therapy but later was readmitted and ultimately died of extensive infiltrates in the kidneys and pancreas. Erdheim-Chester disease, an extremely rare multisystem histiocytic disorder, has been characterized as a lipid (cholesterol) granulomatosis. Renal, cardiac, and pulmonary involvement are noted in most cases; the roentgenographic signs of skeletal involvement, when present, are pathognomonic.

Bone Diseases↗
PubMed1978

[The Solanum molacoxylon: from the toxic plant to the therapeutic agent (author's transl)].

The solanum molacoxylon (SM) is a plant which grows in South America, whose leaves produce a calcinosis on animal farms. In the aquous extract of the leaves a glycoside of 1, 25 (OH)2D3 has been found. The aquous extracts of the leaves of SM provokes an increase of intestinal calcium absorption without any transformation, because it is active in total nephrectomized animal. If this extract is given during a short time the bone resorption is dramatically increased, nevertheless if it is given chronically an increase of bone formation with an osteosclerosis is observed. This plant increases the plasma and urinary calcium and sometimes the plasma phosphorus. In acute administration the urinary hydroxyproline increases. A few therapeutic assays were done in humans with similar results. These assays suggest that SM could be a natural source of 1,25 dihydroxycholecalciferol.

Animals↗