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PubMed1996

Bone density, bone markers and bone radiological features in mastocytosis.

We examined the association between severity of disease in mastocytosis and skeletal manifestation and bone markers in 16 patients varying in extent of mastocytosis as determined by the urine excretion of methylimidazoleacetic acid. Both osteoporosis and osteosclerosis were found. Bone density in the hip was significantly higher (p < 0.05) in both men and women with an enhanced histamine metabolite excretion. Patients with only moderately increased mast cell mass had low bone mineral density in the hip, osteoporosis and vertebral fractures. The different skeletal disease patterns in mastocytosis might be the effect on osteoblasts and osteoclasts of biologically active substances. Mast cells release a number of vasoactive substances, including histamine which promotes osteoblasts and heparin and prostaglandin D2 which induce bone resorption by activation of osteoclasts. Systemic mastocytosis is a rare disease characterized by multi-organ infiltration by mast cells and with varying skeletal manifestations including osteoporosis. Treatment with bisphosphonates may be beneficial in arresting osteoporosis in this disorder.

Acid Phosphatase↗
PubMed2003

Regulation of osteoclast apoptosis by ubiquitylation of proapoptotic BH3-only Bcl-2 family member Bim.

Osteoclasts (OCs) undergo rapid apoptosis without trophic factors, such as macrophage colony-stimulating factor (M-CSF). Their apoptosis was associated with a rapid and sustained increase in the pro-apoptotic BH3-only Bcl-2 family member Bim. This was caused by the reduced ubiquitylation and proteasomal degradation of Bim that is mediated by c-Cbl. Although the number of OCs was increased in the skeletal tissues of bim-/- mice, the mice exhibited mild osteosclerosis due to reduced bone resorption. OCs differentiated from bone marrow cells of bim-/- animals showed a marked prolongation of survival in the absence of M-CSF, compared with bim+/+ OCs, but the bone-resorbing activity of bim-/- OCs was significantly reduced. Overexpression of a degradation-resistant lysine-free Bim mutant in bim-/- cells abrogated the anti-apoptotic effect of M-CSF, while wild-type Bim did not. These results demonstrate that ubiquitylation-dependent regulation of Bim levels is critical for controlling apoptosis and activation of OCs.

Animals↗
PubMed2001

Therapeutic possibilities of long-term roxithromycin treatment for chronic diffuse sclerosing osteomyelitis of the mandible.

The clinical efficacy of long-term roxithromycin treatment was examined objectively in nine patients with chronic diffuse sclerosing osteomyelitis of the mandible. Roxithromycin was administered orally at a dose of 300 mg/day for between 68 days and 66 months. In seven of the nine cases (77.8%), the symptoms disappeared 1-12 months after the start of therapy. Radiography showed that osteolytic changes (evident from 'moth-eaten' appearance of bone) had improved but that osteosclerosis had persisted or become more predominant by the end of therapy. Therefore, the optimum duration of treatment should be decided according to the amelioration of symptoms along with the disappearance of osteolytic findings in radiographs. Diarrhoea and stomach discomfort occurred in one case, and liver dysfunction in another, but these adverse reactions were relatively mild. The mechanism of action of roxithromycin in this study is not yet fully understood, but our results indicate that long-term roxithromycin treatment may be useful for diffuse sclerosing osteomyelitis of the mandible and should be attempted before surgical treatment is considered.

Adult↗
PubMed2002

Erdheim-Chester disease with prominent pericardial involvement: clinical, radiologic, and histologic findings.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis. Herein we describe documented skeletal and pericardial involvement by ECD producing cardiac tamponade in a 30-year-old woman. The diagnosis of ECD was established by histopathology, immunocytochemistry, and by radiologic studies demonstrating diffuse, bilateral, symmetrical osteosclerosis of the long bones, sparing the epiphyses and axial skeleton. Scintography using methyl diphosphonate showed increased uptake in involved bone. The patient presented with jaundice and hepatic congestion produced by cardiac tamponade. Pericardial biopsy revealed xanthogranulomatous lesions comprised of foamy and lipid-laden macrophages, multinucleated giant cells, monocytes, and lymphocytes in a mesh of fibrosis. Immunohistochemical staining was positive for CD68 and negative for CD1a, consistent with ECD rather than with the much more common Langerhans cell form of histiocytosis.

Adult↗
PubMed1981

Treatment of secondary hyperparathyroidism in patients with chronic renal failure by total parathyroidectomy and parathyroid autograft.

Sixty-one patients with chronic renal failure and secondary hyperparathyroidism underwent total parathyroidectomy and parathyroid autograft. Symptoms relieved by parathyroidectomy included bone pain, pruritus, soft tissue calcification, muscle weakness and healing of fractures. Serum parathormone levels measured before and after operation in 48 patients returned to normal in all but two patients. Serum alkaline phosphatase levels also returned toward normal after operation, except in one patient with a retained parathyroid gland. Complete radiographic studies before and after operation were available in 30 of 61 patients. Twenty-three of 24 patients with osteitis fibrosa had evidence of healing, and in one patient no change occurred. Osteosclerosis noticed in 23 patients improved slightly in eight patients, did not change in 14 and became worse in one. Pathologic examinations revealed 45 patients to have diffuse hyperplasia and 16 nodular hyperplasia. There were two early postoperative deaths, in the first 30 days, and 16 late postoperative deaths, from four months to four years afterward. In no case did the operation contribute to death. Some patients required the administration of supplemental calcium after operation, but in no instance did profound hypocalcemia occur. No patient developed recurrent hyperparathyroidism.

Adolescent↗
PubMed1985

Skeletal complications of Gaucher disease.

Gaucher disease is a collection of related disorders of sphingolipid catabolism caused by the deficiency of a specific beta-glucosidase. The inefficiency of this enzyme, glucocerebrosidase, to degrade its natural substrate leads to the accumulation of the complex lipid glucocerebroside in tissue macrophages. The pathogenesis of the disease is, as yet, poorly understood. The manifestations of the disease are protean with hepatosplenomegaly and bone deterioration frequently the predominating signs. The disease most frequently causes disability because of its effects on the skeleton. This review seeks to summarize the current clinical understanding of these complications. Experience with 327 patients reveals that the bone disease in this disorder is extremely variable. The severity of the problems range from asymptomatic persons with neither radiographic, scintigraphic, nor histologic evidence of bone involvement to those whose skeleton is completely devastated by a process of osteopenia, osteonecrosis, and osteosclerosis. These severely affected individuals show the most bizarre deformities in their bones and are subject to pathologic fracture. Most patients fortunately, are less profoundly affected, but many are plagued by bone pain of an arthritic nature or by an acute prostrating bone crisis probably best described as a bone infarction. The accepted etiology that these crises are a result of vascular compromise produced by occlusion of vessels by Gaucher cells is not supported by scintigraphic or histologic studies. Moreover, the vascular hypothesis does not explain the variety of lesions of the skeleton seen in this multifocal bone disease. Preliminary metabolic and endocrinologic studies suggest that this is not a systemic disorder of metabolism which affects bone uniformly. On the contrary, the lesions are multiple and localized, and sometimes much of the skeleton is preserved. These observations suggest that bone is affected because of collections of Gaucher cells scattered throughout its substance and may be the result of a toxic process around these foci. Alternatively, the storage of glucocerebroside in tissue macrophages may disturb the generation of competent osteoclasts and thus result in a failure to maintain a healthy skeleton. Further research is needed to delineate the pathogenesis of this disorder before any effective therapy can be developed.

Adolescent↗
PubMed2001

The Schnitzler syndrome. Four new cases and review of the literature.

The Schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver and/or spleen, elevated erythrocyte sedimentation rate, and leukocytosis. The mean delay to diagnosis is more than 5 years, and this syndrome is of concern to internists and many medical specialists. Patients with this syndrome are often initially considered to have lymphoma or adult-onset Still disease, which are the main differential diagnoses. However, hypocomplementic urticarial vasculitis, systemic lupus erythematosus, cryoglobulinemia, acquired C1 inhibitor deficiency, hyper IgD syndrome, chronic infantile neurologic cutaneous and articular (CINCA) syndrome, and Muckle-Wells syndrome should also be excluded, because diagnosis relies on a combination of clinical and biologic signs and there is no specific marker of the disease. The disease pursues a chronic course, and no remissions have yet been reported. Disabling skin rash, fever, and musculoskeletal involvement are the most frequent complications. Severe anemia of chronic disease is another serious complication. The most harmful complication, however, is evolution to an authentic lymphoplasmacytic malignancy, which occurs in at least 15% of patients. This hematologic transformation can occur more than 20 years after the first signs of the disease, thus patients deserve long-term follow-up. Treatment is symptomatic and unsatisfactory. The skin rash is unresponsive to treatment, and nonsteroidal antiinflammatory drugs, antihistamines, dapsone, colchicine, and psoralens and ultraviolet A (PUVA) therapy give inconstant results. Fever, arthralgia, and bone pain often respond to nonsteroidal antiinflammatory drugs. In some patients, these symptoms and/or the presence of severe inflammatory anemia require steroids and/or immunosuppressive treatment, which ameliorate inflammatory symptoms but do not change the course of the skin rash.

Adult↗
PubMed2000

Xanthoma of the temporal bone: case report.

OBJECTIVE AND IMPORTANCE: Xanthomas of the cranium that are not accompanied by endocrine or metabolic abnormalities are extremely rare. It is very important to understand the pathological features of this disease, for differential diagnosis from other diseases. CLINICAL PRESENTATION: A 62-year-old woman presented to the hospital with headaches. Cranial x-rays revealed bone destruction in the right temporal bone, with osteosclerosis in the area surrounding the lesion. In computed tomographic scans, the bone cortex was intact and the diploë was dilated because of the large mass of the lesion. T1-weighted magnetic resonance imaging findings were heterogeneous, with areas of hypo- and isointensity; T2-weighted magnetic resonance imaging findings were also heterogeneous, with areas of hypo- and hyperintensity. INTERVENTION: Total removal of the tumor was performed, followed by cranioplasty using artificial bone. DIAGNOSIS: Histologically, lipid-containing foamy cells were dense and a cholesterin granuloma was observed. There was no reason to infer other diseases, and a diagnosis of xanthoma of the temporal bone was reached. CONCLUSION: Xanthomatous lesions are observed in various pathological conditions, including malignant diseases. Xanthomas are benign lesions and the prognoses for patients with these lesions are satisfactory, even after partial excision. Therefore, it is quite important to distinguish xanthomas from other diseases that produce xanthomatous lesions.

Diagnosis, Differential↗
PubMed2000

Posterior spinal instrumentation and fusion of a neuromuscular scoliosis in a patient with autosomal dominant osteopetrosis.

STUDY DESIGN: A case report of a patient with autosomal dominant osteopetrosis and neuromuscular scoliosis who required surgical instrumentation and fusion of her spine. OBJECTIVE: To illustrate the surgical technique and long-term outcome in this rare form of spinal deformity. SUMMARY OF BACKGROUND DATA: Osteopetrosis is a group of rare skeletal dysplasias characterized clinically by skeletal osteosclerosis that is classically described in appearance as "marble bone." Despite the ubiquitous involvement of the vertebra, clinical manifestations of spinal involvement are uncommon. We present the case of an osteopetrotic patient with neuromuscular scoliosis who required surgical correction of her progressive deformity. There are no prior reports in the literature concerning operative or nonoperative management of scoliosis in this patient population. METHODS: The surgical technique utilized as well as the patient's response to surgical management of her scoliosis is presented with 5 year follow-up. RESULTS: The patient underwent a successful T4 to L1 posterior spine fusion and instrumentation using Luque rods, sublaminar wires and allograft bone augmentation. At 5 years following her index procedure, she is clinically and radiographically fused. CONCLUSION: Patients with osteopetrosis present unique surgical challenges during surgical correction of spinal deformities. The use of segmental sublaminar wires with 1/4-inch rods and crosslinks afforded stable fixation despite poor bone quality. Allograft bone combined with postoperative bracing resulted in a well-maintained correction and a solid fusion. Five year follow-up and continued radiographic evidence of stable fusion indicate that the presented approach can lead to a successful outcome in the osteopetrotic patient population.

Adult↗
PubMed2003

Further delineation of Raine syndrome.

We report o a baby from an Arab family with Raine syndrome. The baby presented at birth with severe craniofacial anomalies including a wide anterior fontanelle, exophthalmos, severe depression of the nasal bridge with a hypoplastic midface, bilateral choanal atresia and a large protruding tongue. All the limbs were short and the thorax was small. Radiologically there was increased bone density in some bones, periosteal new bone formation and marked bowing of the femurs, tibiae, and ulnae. We suggest that osteosclerosis in Raine syndrome is not necessarily severe and generalized, and bowing of the long bones is another variable radiological feature of the syndrome.

Bone Diseases, Developmental↗
PubMedDate not supplied

The emerging role of bisphosphonates in prostate cancer.

Bisphosphonates are a class of therapeutic agents originally designed to treat loss of bone density. It has been shown that the primary mechanism of action is inhibition of osteoclastic activity. Accumulating data show that these drugs are useful in diseases with propensities toward osseous metastases. In particular, they are effective in diseases in which there is clear upregulation of osteoclastic or osteolytic activity such as breast cancer and multiple myeloma. Despite the fact that osseous metastases in prostate cancer manifest as osteosclerosis rather than osteolysis, studies now show that bisphosphonates are useful in the management of this disease. In particular, they have demonstrated an impact on osteoporosis associated with hormonal therapy, bone pain from metastases, and skeleton-related events from prostatic adenocarcinoma. This review briefly summarizes the available clinical data on the utilization of bisphosphonates in the disease of prostate cancer.

Animals↗
PubMed2003

Prominent Tc-99m MIBI skeletal uptake in renal osteodystrophy: a possible role for whole-body scanning.

Renal osteodystrophy is a process whereby renal failure causes profound bone disease. Effects on bone include osteosclerosis, osteomalacia, osteoporosis, pathologic fractures, aseptic necrosis of the hips, and bone pain. The authors present a case of renal osteodystrophy with intense Tc-99m 2-methoxy isobutyl isonitrile (MIBI) radiotracer uptake in the mandible and propose that Tc-99m MIBI may have a potential role in diagnosing renal osteodystrophy.

Bone and Bones↗
PubMed1985

Polyclonal plasma cell proliferation with systemic capillary hemangiomatosis, endocrine disturbance, and peripheral neuropathy.

An autopsy case of so-called plasma cell dyscrasia (PCD) of a 37-year-old woman was reported. This case showed typical clinical symptoms of PCD, except for no abnormality in serum immunoglobulins. Besides the typical histological findings often observed with PCD, the autopsy revealed an additional finding of systemic angiomatous proliferation of small blood vessels throughout the body, especially in the skin, kidneys, brain, various lymph nodes, and soft tissue. A peculiar glomerular lesion in this case, similar to membranoproliferative glomerulonephritis, was likely to be a result of proliferation of glomerular capillaries. In addition, this case showed histological evidence of retroperitoneal fibrosis and osteosclerosis. These findings were also considered to be due to the vascular abnormality. Although the etiology of the vascular lesions is obscure, it is emphasized that the abnormality is probably an important cause of the clinical symptoms seen in PCD.

Adult↗
PubMed2006

Biliary manifestation of Erdheim-Chester disease mimicking Klatskin's carcinoma.

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis, characterized by infiltration of foamy, lipid-laden histiocytes often affecting the lower extremities and resulting in symmetrical osteosclerosis. Internal organs involved include the lung and the kidneys, reflecting progressive disseminated granulomatous infiltration. Progression may be fatal, especially in cases of systemic extraosseous involvement. Here, we report a patient with elevated serum levels of liver enzymes due to intra- and extrahepatic bile duct stenoses. The patient's past medical history was remarkable for ECD, since 1 yr before he had undergone surgery for a pituitary lesion in our neurosurgical department revealing the typical histological and immunohistochemical criteria of ECD. Because no biliary manifestation of ECD had been described so far in the literature, surgery of suspected bile duct carcinoma was performed unraveling an unresectable tumor of the hilar region. Surprisingly, histologic examination of intraoperative biopsy specimens failed to demonstrate malignancy but rather revealed another xanthogranulomatous lesion embedded in extended periductal fibrosis as is typically described in extrahepatic parenchymal organ manifestation of ECD. Other possible reasons for cholestatic liver disease were excluded. Secondary cholestasis was overcome by endoscopic dilatation and biliary stenting with stents being exchanged every 3 months. During follow-up for 7 yr we have observed only a slight increase of the hilar stenosis so far. This is the first report describing biliary manifestation of ECD. Even though ECD is a rare cause of cholestasis, it should be considered in patients with this disorder in the setting of multiorgan manifestation.

Bile Duct Neoplasms↗
PubMed1993

A panoramatomographic study of the teeth and jaws of Finnish university students.

A random sample of panoramic radiographs was taken of 1027 (386 male and 641 female) university students. All the students were born between 1965 and 1971. The following were examined: caries, apical radiolucencies, endodontically treated teeth, marginal and vertical bone loss, third molars, supernumerary, missing, and persisting teeth, bone structure, condyles, maxillary sinuses and soft tissue calcifications. In the inter- (of two examiners) and intra-examiner variation Kappa indices varied between 0.15 and 1.00, and agreement percentages from 52, to 100. Kappa index was poor (0.15) in evaluation of the bone structure. The mean DMFT index for the students was 8.4. A sound dentition was found in 6.6%, and 48.4% of the students were free of caries. The mean number of carious lesions was 1.1. There were 50 retained roots (49 from primary teeth). 76.6% of the students had one or more unerupted wisdom teeth and 22.7% had radiologic changes typical of pericoronitis in the region of the mandibular third molars. 16.2% of the students had at least one missing wisdom tooth. Bone structure was normal in 92.4% of the students, idiopathic osteosclerosis being the most common divergence (3.5%). Arthrotic changes in the condyles were found in 9% of the students. In eight cases there was erosion of the condyles, which was diagnosed as arthritis. Styloid ligament calcifications were found in 21.5% of the students. In the maxillary sinuses, male students exhibited changes 1.7 times more often than female students (26.3% in males and 15.1% in females).

Adult↗
PubMed1986

Megakaryoblastic transformation of myelofibrosis with expression of the c-sis oncogene.

We describe a case of primary myelofibrosis which terminated in an acute megakaryoblastic leukaemia with massive marrow fibrosis and osteosclerosis. The megakaryocyte lineage of the terminal phase was confirmed by ultrastructural and surface marker studies of the blast cells. The leukaemic phase was associated with the presence of large numbers of progressively more immature megakaryocyte progenitors in the peripheral blood. The expression of c-sis mRNA in these blast cells was significantly higher than in normal mononuclear cells. Activation of the c-sis protooncogene leading to increased production of platelet-derived growth factor could be related to the progressive fibrosis observed.

Adult↗
PubMed1991

Inherited erythrocyte pyruvate kinase deficiency in a beagle dog.

A 1-year-old, female Beagle dog with minimal exercise intolerance was found to have a persistent, severe, and highly regenerative anemia, splenomegaly, and progressive osteosclerosis. Despite near-normal in vitro erythrocyte pyruvate kinase (PK) activity, the authors diagnosed PK deficiency by demonstrating a glycolytic block at the PK step, the lack of normal R-type PK isoenzyme, and the presence of M(2)-type PK in the animal's erythrocytes. The dam had half-normal erythrocyte PK activity, which supports an autosomal recessive mode of inheritance. We conclude from our studies that close similarities exist between erythrocyte PK deficiency in Beagle, Basenji, and West Highland White Terrier dogs and that this form of PK deficiency may be more widespread than previously thought.

Journal Article↗
PubMed2006

Pathogenesis, laboratory diagnosis, and clinical implications of erythrocyte enzyme deficiencies in dogs, cats, and horses.

Deficiencies of enzymes involved in erythrocyte metabolism can have significant effects on erythrocyte function and survival. Animals with pyruvate kinase (PK) or phosphofructokinase (PFK) deficiencies have shortened erythrocyte life spans and regenerative anemia. PK-deficient dogs (but not PK-deficient cats) develop progressive myelofibrosis and osteosclerosis of bone marrow and hemochromatosis and cirrhosis of the liver. PFK-deficient dogs have sporadic episodes of hyperventilation-induced intravascular hemolysis and hemoglobinuria. Cytochrome b5 reductase (Cb5R) deficiency in dogs and cats results in persistent methemoglobinemia and cyanotic mucous membranes. Severe deficiency of glucose-6-phosphate dehydrogenase, the rate-controlling enzyme in the pentose phosphate pathway, resulted in anemia with eccentrocytosis in an American saddlebred colt. Horses with erythrocyte flavin adenine dinucleotide (FAD) deficiency have both eccentrocytosis (attributable to severe deficiency in glutathione reductase activity) and methemoglobinemia (attributable to Cb5R deficiency); the dual enzyme deficiency occurs because FAD is a required cofactor for both enzymes. Erythrocyte enzyme deficiencies do not usually shorten life expectancy, except for PK-deficient dogs and potentially PFK-deficient dogs during a hemolytic crisis. Although enzyme deficiencies are rare causes of anemia and methemoglobinemia, the ability to diagnose deficient animals allows for the possibility of eliminating these undesirable traits in future breeding. DNA-based assays are available for PK and PFK deficiencies; whereas, biochemical tests of enzyme activity are required for other deficiencies. Continued research is needed to document additional enzyme deficiencies that likely occur and to develop additional DNA-based assays to detect heterozygous animals.

Animals↗