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PubMed1990

Myeloproliferative disorders. Classification and diagnostic features with special emphasis on chronic myelogenous leukemia and agnogenic myeloid metaplasia.

Leukocytosis, mild anemia, thrombocytosis, and panhyperplasia in the marrow characterize the early stages of most of the CMPD, whereas extramedullary hematopoiesis (such as in the spleen or liver), peripheral cytopenias (anemia, leukopenia, or thrombocytopenia), and myelofibrosis, with or without osteosclerosis, reflect the changes seen in the later stages. Transitions among the different CMPD and termination in acute leukemia or marrow failure also are common. CML often is characterized by leukocytosis and the presence of the entire spectrum of granulocytes (mature and immature) in the blood and marrow, reduced LAP, hypercellularity with prominent granulocytic hyperplasia in the marrow, Ph chromosome, and bcr-abl gene rearrangement. Typical features of AMM include leukoerythroblastosis, teardrop poikilocytosis, anemia, increased or normal LAP, prominent megakaryocytic hyperplasia in the marrow, dyshematopoiesis, and hyperplastic or fibrotic/sclerotic marrow.

Chromosome Aberrations↗
PubMed1990

Five-year results of bipolar arthroplasty with bone grafts and reamed acetabula for osteoarthritis in young adults.

Bateman universal proximal femoral arthroplasty combined with acetabular reaming was performed in 35 cases of osteoarthritis of the hip joint. The patients were from 21 to 59 years of age (average, 41 years of age) at the time of operation. The postoperative outcome for a middle term ranging from two to eight years, with an average of five years and nine months, was studied along with postoperative migration of the outer head. The clinical outcome, assessed by the evaluation criteria for hip function proposed by the Japanese Orthopaedic Association, was favorable. A stable average score of greater than 80 occurred for eight years postoperatively; the average preoperative score was 45. Of the 35 cases, seven cases (20%) had an upward migration of the outer head, and one case (2.9%) had an inward migration of 5 mm or more, but the migration did not affect the clinical outcome in any case. There was no certain relationship between the degree of rotation of the outer head and the extent of its migration. Migration of the outer head was prevented by postoperative exercise, in which total loading was initiated after the completion of the radiolucent zone around the outer head and its circumferential zone of osteosclerosis.

Acetabulum↗
PubMed1990

[Histology of the cystic area of the ischemic femoral head in the adult].

16 ischemic femoral heads from human adult were studied with radiologically and histologically. The two heads from fresh fracture of femoral neck of 1 month looked grossly normal; radiograms and CT showed no abnormality though necrosis surrounded by living bone tissue without cystic formation was found on the top area histologically. The pathology including the 12 collapsed heads from old neck fracture and 2 osteoarthritic heads were similar but varied in degree and extent of mineralization with time. Numerous cysts, were walled off by a layer of osteosclerosis on the necks side. While dense fibrovascular tissues some containing bits of dead bone trabeculae and cartilage filled certain cystic areas, empty cysts were found in 8 of the specimens. Beyond the cystic area, the bone and bone marrow adequately congested and had fairly good blood supply. Based on these findings, the authors suppose that leaking of joint fluid through fissures into deeper part of the head form cisterns which expand with increasing joint fluid pressure may be one of the causes of cyst formation. In early stage of ischemia with head in normal contour, replacement of the degenerated area with bone grafting may serve as a good choice of treatment.

Cysts↗
PubMed1989

[A case of breast cancer with multiple bone metastases effectively treated with UFT and tamoxifen].

A 51-year-old female with advanced breast cancer involving multiple bone metastases was daily administered 400 mg of UFT and 20 mg of tamoxifen. X-ray examination revealed remarkable osteosclerosis. Laboratory data revealed a high serum alkali phosphatase level of 820 mU/ml(at 3 months), which gradually decreased to 274 mU/ml with pain relief at 11 months after the beginning of this chemotherapy.

Alkaline Phosphatase↗
PubMed1989

[Experimental studies on haematogenous osteomyelitis due to Staphylococcus hyicus in mice].

10(7) colony forming units of 18 different strains of Staphylococcus hyicus were intravenously administered into mice. Five mice as a group received each strain of S. hyicus. At 14 days after inoculation, 11 out of 18 strains were found in the bone marrow. Out of these 11 strains, SMUH 10 and SMUH 18 caused remarkable inflammation which was characterized by roentgenographical change. A number of these organisms were detected in the bone marrow until 28 and 42 days after the injection, suggesting specific lodgement of the strain in mouse bone marrow. X-rays of the upper and lower extremities taken 14 days after inoculation showed multiple small radiolucent areas surrounded by small rims representing osteosclerosis. However, these changes did not show any aggravation during 70 days after inoculation suggesting tendency of improvement.

Animals↗
PubMed1989

[Review of 115 cases of osteoid osteoma].

115 cases of osteoid osteoma treated over more than 18 years is reported. In 1 case the nidus was manifested in 2 different sites after 14 years. The following is emphasized: the terminology for osteoid osteoma should be changed in reference to osteoblastoma; osteoid osteoma and osteoblastoma constitute different aspects of the same neoplasia; the number of observations of osteoid osteoma increases as there is greater knowledge on the varieties of the tumor; treatment, which is exclusively surgical, may be limited to excision of the nidus, in order to guarantee the best healing possible. Resection should be limited to observations where the nidus is masked by osteosclerosis.

Adolescent↗
PubMed1989

[Morphometric study of bone in domestic pigs with fluorosis caused by coal-burning pollution].

Forty four domestic pigs were divided into 2 groups: control group: 17 pigs; fluorosis group: 27. They were fed for 14 months in the endemic area or non-endemic area respectively. In some of the pigs, double tetracycline was given for bone dynamic study, and blood and urine were collected for biochemical analyses. Iliac, tibia, fibula, the 2nd metatarsal and the 2nd vertebrae were reserved either for bone density measurement or for bone histomorphometric study. The results showed that fluoride content either in serum and in urine or in bone tissue was significantly elevated. The bone density of tibia was increased. Bone histomorphometry showed that trabecular bone volume of iliac and vertebrae was increased while that of fibula head was decreased. Trabecular resorption surface of fibula head was expanded while that of vertebrae was shrunk. As to bone dynamics, both bone formation rate and bone mineralization rate were inhibited, indicating that the toxic effects of fluoride in tremendous amount on bone remodeling. The authors suggest that the existence of osteosclerosis of axial and osteoporosis of peripheral bone in fluorosis might be related to the redistribution of calcium within the body. Furthermore, fluoride may be an osteomalacic factor for the development of osteomalacia in endemic fluorosis.

Air Pollution↗
PubMed1989

[Therapeutic effect of low-fluorine drinking water on fluorosis of bone].

495 clinical cases and 25 radiographic cases of fluorosis of bone diagnosed six years ago in an endemic area showed satisfactory results after six years' treatment with low-fluorine drinking water. Among these, 140 have been completely cured, 105 have gained marked clinical improvement and 172 have gained moderate improvement; the cure rate being 49.49% and the effective rate, 84.84%. Of the 25 X-ray diagnosed cases, 15 showed marked improvement. Radiographic examination has revealed reappearance of evenly distributed fine bone trabeculae. Two cases of grade III osteosclerosis have turned to grade II; 4 to grade I; 8 cases of grade II have turned to grade I; 1 case of grade I has become normal. The results demonstrate that fluorosis of bone is reversible.

Adult↗
PubMed1989

[Bilateral angiomyolipomas of the kidney in Bourneville's tuberous sclerosis].

We report on a 25-year-old patient diagnosed as having Bourneville tuberous sclerosis with a giant angiomyolipoma 16 X 12 cm. in diameter, and two small angiomyolipomas in the left kidney, multiple asymptomatic angiomas in the right kidney and two 1 cm. diameter angiomas in the liver. The presenting features were intense left-sided abdominal pain of sudden onset, frank hematuria, acute anemic syndrome, nausea and vomiting. Patient work up included x-ray of chest, abdomen, and skull, bilateral renal arteriography and ultrasound were diagnostic of Bourneville tuberous sclerosis with multiple bilateral renal angiomyolipomas. The hematologic and hemodynamic compromise and the almost completely absent intact parenchyma in her left kidney did not permit a conservative surgical procedure and the patient was submitted to a left nephrectomy. The patient had the following characteristic features of Bourneville's disease: epilepsy, intracranial calcifications on CT, sebaceous adenomas on face, fibromas under nails of left hand and foot, bony lesions in the form osteosclerosis and chylous pleural effusion. Her intelligence level was normal and no optic nerve phakomas were observed. The literature on angiomyolipoma and Bourneville's tuberous sclerosis is reviewed. The importance of using ultrasound and CT in combination to diagnose and follow up renal angiomyolipomas is highlighted. Treatment of angiomyolipomas must be based on two parameters: symptoms and size. Symptomatic angiomyolipomas warrant angiographic work up and selective arterial embolization or the most conservative surgical procedure possible (enucleation, partial nephrectomy). Asymptomatic angiomyolipomas warrant CT and/or ultrasound examination every 6 or 12 months depending on size.

Adenoma↗
PubMed1989

Osteoarthritis associated with osteopetrosis treated by total knee arthroplasty. Report of a case.

Osteopetrosis is due to a defect in osteoclastic cell function and results in osteosclerosis and progressive obliteration of the marrow spaces. Early onset osteoarthritis is associated with osteopetrosis. The authors describe a case of early onset osteoarthritis associated with osteopetrosis treated by total knee arthroplasty. The marblelike quality of osteopetrotic bone makes surgical treatment of these patients technically challenging and requires modification of standard surgical technique.

Female↗
PubMed1986

[Special radiology of the hand in renal osteopathy].

The different radiographical abnormalities of the hand with renal osteodystrophy are demonstrated. The soft tissue immersion low-energy X-ray technique allows early detection of macrostructural changes of spongy and compact bone, irregularities of the cortex, defects and pseudocysts, osteosclerosis as well as calcifications in the soft tissue and arterio sclerosis of patients with chronic renal failure. Possible erroneous interpretations will be discussed.

Arthrography↗
PubMed1988

[Biologico-mechanical behavior of bone in osteomalacia and in phosphate diabetes in adulthood].

The main orthopedic complications in osteomalacia, skeletal deformities, Looser's transformation zones and fractures, are described. The limited value of a Looser's transformation zone as a radiographic diagnostic criterion for osteomalacia is mentioned, because such zones can also occur in other bone diseases with abnormal remodeling. This paper also outlines the differential diagnosis of a Looser zone against a stress fracture. In osteomalacia and phosphate diabetes the biological and mechanical properties of bone are different. In adult phosphate diabetes radiological examination is more likely to reveal osteosclerosis than a radio-opaque bone. For this reason we investigated the bone healing process in five adult patients with osteomalacia and in six with phosphate diabetes. In spite of the small number of patients we may assume that bone healing after surgery is delayed in phosphate diabetes compared with osteomalacia, in which a normal bone healing tendency is present.

Adult↗
PubMed1977

IgE myeloma with osteoblastic lesions.

A 69-yr-old man with persistent anemia had multiple myeloma with an IgE-type kappa M component and Bence Jones proteinuria. Bone x-rays revealed occasional lytic lesions associated with a diffuse sclerotic reaction throughout the skeleton. Special bone histologic studies utilizing tetracycline labeling, undercalcified sections, and microradiography confirmed active osteoblastic activity. This case was compared with the four previously reported cases of IgE myeloma, one of which also had osteosclerosis.

Aged↗
PubMed1986

Skeletal fluorosis in humans: a review of recent progress in the understanding of the disease.

Endemic skeletal fluorosis is a chronic metabolic bone and joint disease caused by ingesting large amounts of fluoride either through water or rarely from foods of endemic areas. Fluoride is a cumulative toxin which can alter accretion and resorption of bone tissue. It also affects the homeostasis of bone mineral metabolism. The total quantity of ingested fluoride is the single most important factor which determines the clinical course of the disease which is characterized by immobilization of joints of the axial skeleton and of the major joints of the extremities. A combination of osteosclerosis, osteomalacia and osteoporosis of varying degrees as well as exostosis formation characterizes the bone lesions. In a proportion of cases secondary hyperparathyroidism is observed with associated characteristic bone changes. Contrary to earlier thinking, severe crippling forms of skeletal fluorosis are seen in paediatric age group too. Increased metabolic turnover of the bone, impaired bone collagen synthesis and increased avidity for calcium are features in fluoride toxicity. Osteosclerotic picture is evident when small doses of fluoride are ingested over a long period of time during which calcium intakes are apparently normal while osteoporotic forms are common in paediatric age group and with higher body load of the element. Alterations in hormones concerned with bone mineral metabolism are seen in fluorosis. Kidney is the primary organ of excretion for fluorides. Age, sex, calcium intake in the diet, dose and duration of fluoride intake and renal efficiency in fluoride handling are the factors which influence the outcome. Serum parameters rarely help in the diagnosis. Elevated urinary fluoride and increased bone fluoride content are indicators of fluoride toxicity. Fluorosis is a preventable crippling disease. No effective therapeutic agent is available which can cure fluorosis. Industrial fluorosis is on the increase on a global basis. Bone density measurement is a tool for early diagnosis.

Bone Diseases↗
PubMed1988

[Idiopathic myelofibrosis. Radiological aspects of bone changes].

Idiopathic myelofibrosis is a chronic myeloproliferative disease characterized by skeletal lesions (30 to 70% of cases). We considered 49 patients with idiopathic myelofibrosis treated between 1972 and 1986 at the Institutes of Hematology and Radiotherapy, University of Bologna. Only 19 of these patients underwent roentgenographic skeletal surveys, associated with whole body bone scintigraphy in 4 cases, and with CT in 1 case. The most common bone change, as seen in 12 patients, was osteosclerosis, following two distinct patterns: pure, and mixed. Other types of bone involvement (osteoporosis and pure osteolysis) were seen in 2 cases only; in 5 patients radiological skeletal examinations did not show meaningful lesions. Conventional radiology is hardly ever conclusive in the diagnosis of idiopathic myelofibrosis. This is due partly to the often moderate degree of bone involvement, partly to the scanty specificity of the findings. However, a careful examination of the plain roentgenographs, completed when possible by other more recent imaging techniques, may be very important towards a more precise definition of the disease and, in some cases, for a correct diagnosis.

Adult↗
PubMed1986

The osteodystrophy of hypervitaminosis D: a metabolic study.

A patient received 2.5 mg vitamin D2 daily for 10 years and presented with increasing skeletal pain and hypercalcaemia. The limbs were painful to touch especially at the insertions of ligaments and tendons, and radiographs showed osteosclerosis with calcification in the periosteum, blood vessels, tendoachilles and plantar fascia. Bone histomorphometry showed increased amounts of osteoid and defective mineralisation despite hypercalcaemia, hyperphosphataemia and raised serum concentrations of vitamin D metabolites. A negative external calcium balance was documented in the presence of enhanced intestinal calcium absorption and an increase in urinary hydroxyproline excretion. Cortisone improved calcium balance and corrected the hypercalcaemia by reducing serum 1,25-dihydroxyvitamin D levels and urinary hydroxyproline excretion.

Bone and Bones↗
PubMed1986

[Histomorphometric profile of bone fluorosis induced by prolonged ingestion of Vichy Saint-Yorre water. Comparison with bone fluorine levels].

Nine transiliac bone biopsies from 7 patients with skeletal fluorosis due to prolonged ingestion of often high quantities of Vichy Saint-Yorre water were analyzed. Four of these patients also suffered from a chronic renal failure. A histomorphometric study was possible in 8 out of the 9 biopsies. The measurement of bone fluoride content, and a microradiographic examination, were performed on all bone samples. The radiologically evident osteosclerosis observed in each patient was confirmed by the significant increase of trabecular bone volume. Furthermore, the osteoid surfaces were very extended but the thickness of osteoid seams was normal in 6 out of 8 cases. Two biopsies demonstrated a morphological evidence of osteomalacia with abnormally thick osteoid seams. Calcification rate, measured in one of these 2 cases after tetracycline double labeling, was extremely low (less than 0.20 micron/d). The bone fluoride content was significantly high in each specimen (greater than 0.40 bone ash%) and correlated with relative osteoid volume (r' = 0.91) and thickness index of osteoid seams (r' = 0.83). Histologically, bone tissue showed modifications classically reported in the various types of skeletal fluorosis (formation defects, mottled bone with mottled periosteocytic lacunae). In conclusion, the prolonged administration of Vichy Saint-Yorre water containing 8.5 mg of fluoride ion per liter, provokes a skeletal fluorosis. This intoxication appeared very quickly if the patient suffered from an even mild renal failure. Once again, it is shown that a disturbed renal function predisposes to an excessive retention of fluoride.

Adult↗
PubMed1987

Adult osteopetrosis: study of two brothers.

Bone biopsies from 2 adult cases of benign osteopetrosis have been studied histologically and under the electron microscope. The most important findings were a high degree of osteosclerosis, narrowing and fibrosis of medullary spaces, and the presence of abundant uncalcified osteoid tissue with an osteomalacia-like appearance. The osteoclasts were not numerous. All of them lacked the brush border and many showed wide cytoplasmic vacuoles containing amorphous and filamentous material. This picture, which is not different from that found in cases of fetal osteopetrosis, has not been modified by ethydronate therapy.

Biopsy↗