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At least 721 records · Page 40Linked to original sources
PubMed1992

Eighth cranial nerve dysfunction in hyperostosis cranialis interna.

Hyperostosis cranialis interna is a recently described autosomal dominant bone disorder characterised by hyperostosis and osteosclerosis confined to the skull, especially the calvarium and the skull base. In the affected family members, we found variable simultaneous involvement of cranial nerves I, II, VII and VIII from late childhood onwards, most likely due to nerve entrapment. Auditory and vestibular functions were followed in 3 young family members for 8 years. At the first examination, pure tone audiograms were normal in all 3 cases and case 1 showed no caloric response in the right ear. During follow-up, this ear developed severe hearing loss progressing to deafness. The left ear showed transient sensorineural hearing loss and a temporarily diminished caloric response. Similar observations were made in case 2. Both cases showed abnormal brain stem auditory-evoked responses during and after the sudden hearing loss, in which initially only wave I was preserved and later on wave V returned with significantly prolonged I-V interval. The latter phenomenon was also observed in case 3 on both sides in the presence of normal audiograms during and after transient unilateral facial nerve paralysis, which was accompanied by bilateral diminished caloric responses.

Adolescent↗
PubMed1988

Thyroid hormone resistance in blood monocyte cells and elevated serum T3 in patients with autosomal dominant osteopetrosis.

Seven patients with autosomal dominant osteopetrosis from three different families were investigated. They all had the roentgenological type I disease, characterized by universal, symmetrical osteosclerosis and enlarged thickness of the calvarium vault. Serum concentrations of thyroid hormones and the specific nuclear binding of triiodothyronine (T3) in mononuclear blood cells were studied. All patients appeared clinically euthyroid. The serum level of T3 was significantly elevated (serum T3 = 1.89 nmol/l) compared with normal age and sex-matched controls (serum T3 = 1.44 nmol/l, p less than 0.05). The specific maximal nuclear binding capacity of T3 was significantly decreased (MBC = 0.51 fmol T3/mg DNA) in these patients compared with controls (MBC = 1.8 fmol/mg DNA, p less than 0.05) whereas no difference in the equilibrium association constant (Ka) was observed. The decreased specific nuclear binding of T3 and the slightly elevated serum level of T3 might indicate a modest peripheral resistance to T3 in patients with autosomal dominant osteopetrosis type I.

Adult↗
PubMedDate not supplied

Acute lymphoblastic leukemia with severe skeletal involvement: a subset of childhood leukemia with a good prognosis.

Skeletal radiographic abnormalities are common in children with acute lymphoblastic leukemia (ALL). The impact of severe skeletal involvement (SI) on survival and the correlation between SI and biological markers were analyzed. Therefore, radiographs and medical charts of 106 ALL patients who received a skeletal survey at the time of diagnosis and were treated at the University Children's Hospital Würzburg between 1974 and 1995 were reviewed. On the basis of the skeletal survey, SI was quantified using a score. Fifty-nine patients (55%) showed radiographic abnormalities defined as metaphyseal banding (48%), periosteal reactions (11%), osteolysis (33%), osteosclerosis (31%), or osteopenia (22%). Children with severe SI (n = 32) presented with a higher rate of severe radiographic abnormalities such as geographic destructive osteolysis (37%; P < .001) and periosteal reactions (28%; P < .05) compared with children with moderate SI (0% and 4%, respectively). Patients with severe SI showed a lower peripheral blast count (P < .05) at diagnosis, a more frequent "prednisone good response" (P < .05), and a higher survival rate (83 +/- 7%; P < .05) than patients without SI (54 +/- 9%). Patients with moderate SI (n = 27) showed a higher hemoglobin concentration (P < .05), an enlargement of liver (P < .05) and spleen (P < .01), a higher BFM risk factor (P < .01), but still a higher survival rate (73 +/- 11%) than patients without SI (NS). Patients with severe SI had a higher (P < .001) DNA content of leukemic cells as measured by DNA index (DI) than patients without SI. Thirty-one percent of patients with severe SI, 22% of patients with moderate SI, and no patient without SI had a DI > 1.16. No patient with a DI < 1.0 presented with severe SI. The number of radiographic abnormalities in patients with SI correlated with the DI (rho: 0.46; P < .001). However, patients with euploidy (DI = I) and severe SI also had a higher (P = .05) survival rate (70 +/- 18%; n = 15) than euploid patients without SI (49 +/- 11%; n = 24). Of the patients with severe SI, 78% had common ALL and 22% had an ALL type other than common ALL (P < .05). In patients with ALL types different from common ALL, severe SI was also associated with higher survival rates. We conclude that on the basis of clinical features, two distinct subgroups could be identified in terms of SI. Patients with clinically relevant severe SI had a better prognosis, a higher DI, and more frequently a common ALL than patients without SI. However, the impact of severe SI on prognosis was independent of DI and type of leukemia.

Adolescent↗
PubMed1994

Chronic myelomonocytic leukemia associated with primary amyloidosis.

A case of chronic myelomonocytic leukemia (CMML) associated with primary amyloidosis (AL) is presented. Hepatosplenomegaly, macroglossia, and xanthelasma were the major physical findings. Laboratory tests showed macrocytic anemia, thrombocytopenia, monocytosis and a bi-clonal gammopathy. Early monocytes and monoblasts were noted in the bone marrow aspiration biopsy. Cytogenetic evaluation showed a clonal deletion of chromosome 21 long arm (21q-). Amyloid was present in the liver, tongue and xanthelasma. In addition, the patient was noted to have osteosclerosis of the lower extremities. Treatment with prednisone and colchicine resulted in a subjective response. The unusual association of CMML, and primary amyloidosis is discussed.

Adult↗
PubMed1995

Extramedullary megakaryoblastic tumors following an indolent phase of myelofibrosis.

A 59-year-old man developed multiple subcutaneous and bone tumors after 2 years' duration of indolent myelofibrosis. These tumors exhibited a diffuse proliferation of pleomorphic blast cells. The skeletal x-rays showed disseminated osteolysis and osteosclerosis. Blast cells obtained from the pleural effusion did not react with myeloperoxidase, sudan black B or non-specific esterase. Genotypic analysis of DNA samples at autopsy did not demonstrate rearrangements of T-cell receptor beta-chain or immunoglobulin heavy-chain genes. Frozen-section immunohistochemical studies revealed that the neoplastic cells were derived from megakaryocytic lineage. The present case represents a novel and unusual subtype of megakaryocytic neoplasm.

Bone Neoplasms↗
PubMed1976

The subchondral bone in osteoarthritis and rheumatoid arthritis of the knee. A histological and microradiographical study.

Specimens of the joint surfaces of the tibia from patients with OA and RA were exposed were examined for bone mineralization, bone formation, osteoid tissue and bone resorption. Judging from the appearance of the osteoblasts in OA the sclerotic changes are mainly focal with relatively little osteogenesis. No osteoclasia was seen in the sclerotic areas. Breakdown of the mineralized cartilage is followed by the development of cysts with highly cellular connective tissue with high osteoblastic activity and osteoclasia. Osteoid tissue is relatively sparse. The changes in RA are more diffuse with a more active osteoblastic activity and widespread zones of osteoid tissue as well as resorption by osteoclasts. It appears as if the increased uptake of 85Sr in OA is more dependent on the occurrence of relatively inert osteosclerosis than on a rapid turnover of the bone tissue.

Arthritis, Rheumatoid↗
PubMed2002

Osteoclast dysfunction in the osteosclerotic variant of renal osteodystrophy.

Six iliac crest bone biopsies were obtained from five patients with renal osteodystrophy and osteosclerotic lesions to investigate the mechanism of osteosclerosis in renal osteodystrophy. Each specimen was prepared in an undecalcified fashion and measured under light microscopy for routine analysis and histomorphometry. Osteoclast resorptive lacunae were estimated by measuring the lacunar length and depth. Histomorphometric studies revealed an increase in all parameters with a mean trabecular bone volume of 66% (normal: 19%-26%), mean trabecular osteoid surface of 71% (normal: 16%-22%), and a mean trabecular osteoid volume of 15% (normal: 1%-2%). In one patient, there was a ten-fold increase over the norm of osteoclasts/mm2 (0.3; normal: 0.02-0.04), whereas the mean width of trabecular bone was twice the norm. In all biopsies, the calculated lacunar area was markedly diminished compared to published controls. These results support the hypothesis that osteoclast function is impaired in patients with renal osteodystrophy.

Adult↗
PubMed2006

Monocyte/macrophage dysfunctions do not impair the promotion of myelofibrosis by high levels of thrombopoietin.

Several lines of evidence indicate that the megakaryocyte/platelet lineage is crucial in myelofibrosis induction. The demonstration that NOD/SCID mice with functionally deficient monocytes do not develop fibrotic changes when exposed to thrombopoietin (TPO) also suggests an important role for monocyte/macrophages. However, in this animal model, the development of myelofibrosis is dependent on the level of TPO. This study was conducted to investigate whether NOD/SCID mice exposed to high TPO levels mediated by a retroviral vector would be refractory to the development of bone marrow fibrosis. We show that TPO and TGF-beta1 in plasma from NOD/SCID and SCID mice engrafted with TPO-overexpressing hemopoietic cells reach levels similar to the ones reached in immunocompetent mice, and all animals develop a myeloproliferative disease associated with a dense myelofibrosis at 8 wk posttransplantation. Monocytes in NOD/SCID mice are functionally deficient to secrete cytokines such as IL-1alpha in response to stimuli, even under TPO expression. Surprisingly, the plasma of these mice displays high levels of IL-alpha, which was demonstrated to originate from platelets. Together, these data suggest that completely functional monocytes are not required to develop myelofibrosis and that platelets are able, under TPO stimulation, to synthesize inflammatory cytokines, which may be involved in the pathogenesis of myelofibrosis and osteosclerosis.

Animals↗
PubMed2004

Durable responses to thalidomide-based drug therapy for myelofibrosis with myeloid metaplasia.

OBJECTIVE: To present the results of a long-term analysis of 2 sequential phase 2 trials of thalidomide (alone or in combination) for palliation of myelofibrosis with myeloid metaplasia (MMM). PATIENTS AND METHODS: We analyzed (March 1999 to August 2003) initial and long-term outcomes from 36 patients with symptomatic MMM who had enrolled in either our thalidomide single-agent trial (n=15) or our trial of low-dose thalidomide (50 mg/d) combined with prednisone (n=21). RESULTS: Among the 36 study patients, 20 (56%) showed some improvement in their clinical course. Response rates for specific end points included improvements in anemia (15 of 36 [42%]), thrombocytopenia (10 of 13 [77%]), or splenomegaly (5 of 30 [17%]). The combination of low-dose thalidomide and prednisone, as opposed to single-agent thalidomide, was better tolerated and more efficacious. After a median follow-up of 25 months (range, 20-56 months), 10 of 36 patients (28%) showed an ongoing response, including 8 patients in whom protocol treatment has been discontinued for a median of 21 months (range, 16-31 months). Durable treatment responses were documented for only anemia and thrombocytopenia. Treatment response was not affected by the baseline status of bone marrow fibrosis, angiogenesis, osteosclerosis, cytogenetics, or circulating myeloid progenitor (CD34) cell count. Unusual drug effects, all reversible, included leukocytosis (8 patients) and/or thrombocytosis (6 patients). CONCLUSIONS: Thalidomide (alone or combined with prednisone) is an effective first-line treatment of symptomatic anemia or thrombocytopenia in MMM. Thalidomide-based therapy has the potential to produce durable responses in MMM-associated cytopenias, even after discontinuation of the drug.

Adult↗
PubMed2004

Maxillary brown tumor and uremic leontiasis ossea in a patient with chronic renal insufficiency.

Findings of renal osteodystrophy in cranial bones are not uncommon and include osteomalacia, osteosclerosis, erosion of the cortical bone, brown tumors and resorption of the lamina dura. However, massive thickening of the cranial vault and facial bones, called uremic leontiasis ossea, have been reported very rare. In the present article, we describe the case of an uncooperative female patient with a brown tumor, involving the left maxillary sinus and massive thickening of the cranial vault and facial bones, secondary to severe secondary hyperparathyroidism during 8 years of regular hemodialysis treatment.

Adult↗
PubMed2003

Erdheim-Chester disease: a unique presentation with liver involvement and vertebral osteolytic lesions.

Erdheim-Chester disease is a very rare xanthogranulomatous, non-Langerhans cell systemic histiocytosis with an unknown etiology and pathogenesis. Histologically, it is characterized by a diffuse infiltration with large, foamy histiocytes, rare Touton-like giant cells, lymphocytic aggregates, and fibrosis. The histiocytes differ from the Langerhans cell group in ontogenesis, immunohistochemistry (positive for CD68 and negative for CD1a and S100 protein), and ultrastructural appearance (lack of Birbeck granules). Although most of the cases have symmetric osteosclerosis of the long bones, an involvement of the axial skeleton has also been described. Extraskeletal lesions are present in more than 50% of the patients and may involve the retroperitoneal space, lungs, kidneys, brain, retro-orbital space, and heart. This study presents the case of a patient with Erdheim-Chester disease with vertebral destruction and, for the first time, to our knowledge, involvement of the liver. The diagnosis is based on radiologic, histologic, immunohistochemical, and ultrastructural findings.

Adult↗
PubMed1986

Accelerated bone formation causing profound hypocalcemia in acute leukemia.

A patient with acute monocytic leukemia and fibrosis presented with severe hypocalcemia producing tetany, myocardial failure, and ventricular tachycardia with torsades de pointes configuration. Hypophosphatemia, hypomagnesemia, an elevated alkaline phosphatase level, and osteosclerosis were also present. Bone marrow biopsy samples showed fibrosis and thickened bony trabeculae lined with large osteoblasts. Tetracycline labeling showed an increased rate of calcification. Complete remission of the leukemia and fibrosis was achieved with a single 3-week course of low-dose cytarabine and hydroxyurea, with resolution of the hypocalcemia and hypophosphatemia. Calcitriol and etidronate disodium were also administered. The calculated left ventricular ejection fraction increased from 15% to 55% with correction of the hypocalcemia. The hypocalcemia and hypophosphatemia in this patient probably resulted from accelerated bone formation stimulated by the leukemic cells. The high dose of calcitriol that this patient received may have contributed to the remission of the leukemia.

Aged↗
PubMed1976

[Agnogenic myeloid metaplasia (author's transl)].

The AA., present a case of "agnogenic myeloid metaplasia" (MMA). They describe the principal findings that have led to this diagnosis: Fe59-erythrocynesis, radiologic study of skeleton and cytogenetic study. Fe59-erythrocynesis have shown an hypergetting of spleen and liver and an hypogetting of skeleton. The radiography of skeleton and particularly of humerus, femur and pelvis have shown a diffuse osteosclerosis with rare osteolytic areas. The cytogenetic study have shown an alteration with C-group trisomy.

Bone and Bones↗
PubMed2000

"Coated aorta": a new sign of Erdheim-Chester disease.

Erdheim-Chester disease is a rare, non-Langerhans cell form of histiocytosis characterized by osteosclerosis of the metaphyseal regions of long bones, diabetes insipidus, proptosis, and retroperitoneal fibrosis. The latter usually involves the perirenal area and leads to hydronephrosis. Periaortic fibrosis is less frequent. We describe 3 unusual cases of Erdheim-Chester disease with periaortic fibrosis involving the whole aorta and leading to a "coated aorta" appearance on computed tomography scans. Faced with such a singular "coated aorta," bone scintigraphy can be very helpful when searching for Erdheim-Chester disease.

Aortitis↗
PubMed2000

[Radiotherapy of skeletal metastases].

INTRODUCTION: Patients with skeletal metastases represent a large cohort in clinical oncology, and the single most frequent indication for palliative radiotherapy. Patients with cancer of the breast, lung, prostate and those with myelomatosis, constitute approximately 80% of this group. MATERIAL AND METHODS: This paper summarizes data from relevant published clinical trials employing external irradiation for painful skeletal metastases. More recent randomised trials support the view that a single radiation dose of 8-10 Gy is equally efficient as ten treatments of 3 Gy delivered over two weeks. However, some still believe that fractioned regimes to a higher total dose provide better pain relief of a longer duration than a single fraction. RESULTS: We review the current diagnosis and treatment of patients with skeletal metastases and discuss some aspect of tumour biology. The etiology of pain and the pathogenesis of tumour cells affecting bone tissue, resulting in osteolysis and/or osteosclerosis, are discussed. Associated leukocyte-derived osteoclast-activating cytokines that stimulate pain receptors locally, can in part explain why radiotherapy gives such rapid pain relief. INTERPRETATION: The aims of radiotherapy must be assessed in relation to the life expectancy of the patient. Based on actual publications and own experiences, we suggest treatment with 8 Gy x 1 for the majority of patients, and reserve 3 Gy x 10 for patients with longer life expectancy. Both regimes allow retreatment, if and when pain eventually reoccur in previously irradiated areas.

Bone Neoplasms↗
PubMed2000

Renal osteodystrophy in Ramathibodi Hospital: histomorphometry and clinical correlation.

UNLABELLED: The spectrum and clinical relevance of renal osteodystrophy in Thai dialysis patients are unknown. A study was conducted on the prevalence and clinico-pathological correlation of renal osteodystrophy in chronic dialysis patients who attended Ramathibodi Renal Transplant Clinic between September 1996 and March 1998. All possible volunteers were enrolled irrespective of musculoskeletal symptoms. Fifty six dialysis patients, including 17 (30.4%) CAPD and 39 (69.6%) hemodialysis patients, participated in this study. Serum calcium, phosphate, iPTH, and bone specific alkaline phosphatase were determined. Transiliac crest bone specimens were measured with an average of 30 fields/specimen by a specific computer program for bone histomorphometry (Osteomeasure), and were also studied for dynamic by double tetracycline label. Bone mineral density (BMD) was also determined by DEXA scan. The type of bone pathology was based on Fournier's criteria for renal osteodystrophy. The mean +/- SEM for age was 45.52 +/- 1.74 years, dialysis duration 42.26 +/- 5.54 (range 1-156) months, calcium phosphate product 52.31 +/- 2.77, and iPTH 307.73 +/- 62.04 pg/ml. The following types of renal osteodystrophy were found: adynamic bone 23 (41.1%), hyperparathyroid 16 (28.6%), mixed type 11 (19.6%), mild lesion 3 (5.4%), osteomalacia 2 (3.6%), and osteosclerosis 1 (1.8%) cases. Two cases of aluminum related bone disease were found. The distribution of different bone diseases was not affected by mode of dialysis or vitamin D supplement, but it was affected by dialysis duration. High turnover bone diseases were associated with longer dialysis duration (63.19 +/- 8.9 vs 23 +/- 4.73 months), higher iPTH (541.53 +/- 109.32 vs 87.77 +/- 15.76 pg/ml), and higher bone specific alkaline phosphatase (25.43 +/- 5.04 vs 9.62 +/- 1.34 mg/ml) when compared to low turnover bone diseases, p < 0.05. Intact PTH of greater than 200 pg/ml was a good predictor for high turnover bone diseases (74% sensitivity and 96% specificity). BMD at torch and wards areas varied inversely with dialysis duration (r = -0.3 and r = -0.4, respectively; p < 0.05). Chronic dialysis patients had a greater tendency of bone loss compared to the general Thai population. There was no difference in BMD between CAPD and hemodialysis patients or different types of bone lesions. CONCLUSION: Significant bone diseases are common among Thai chronic dialysis patients. Adynamic bone disease is the most common bone lesion followed by hyperparathyroid and mixed type. The spectrum of bone diseases is affected mainly by dialysis duration. Intact PTH is a good predictor of high turnover bone disease. Greater bone loss than in the general population is common in our patients and is also accentuated by longer dialysis duration.

Absorptiometry, Photon↗
PubMed2000

Pyknodysostosis--a report of two cases with a brief review of the literature.

Pyknodysostosis is a rare sclerosing bone disorder that has an autosomal dominant trait. It is characterized by short stature, brachycephaly, short and stubby fingers, open cranial sutures and fontanelle, and diffuse osteosclerosis, where multiple fractures of long bones and osteomyelitis of the jaw are frequent complications. We present a report of two cases of pyknodysostosis with evidence of long bone fractures and chronic suppurative osteomyelitis of the jaws in one of the cases. Some of the specific oral and radiological findings that are consistent with pyknodysostosis are reported, along with a brief review of the literature.

Adult↗
PubMed1975

[The pycnodysostosis (author's transl)].

Two own observations of pycnodysostosis initiated our description of this disease. Inherited as an autosomal recessive trait the syndrome is characterized by a medium degree generalized osteosclerosis, open craniial sutures and fontanels until adult lige, hypoplastic mandibles with deficiency of the angle, prominence of the forehead and occiput, hypoplasia of the paranasal sinuses, commonly observed dysproportionate dwarfism, acro-osteolysis and increased bone fragility. Contrary to osteopetrosis the prognosis is good.

Adolescent↗