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 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There are probably 2.5 million patients with Paget's disease in the U.S.; 125,000 of these have severe disease meriting specific treatment. While the diagnosis can often be made by inspection, or by measurement of the temperature of involved limbs, it is often missed. Nonspecific findings include pain, headaches, deafness, heart failure, neurologic deficits and renal stones. A specific diagnosis can usually be established by radiologic examination of the skeleton and measurement of the serum alkaline phosphatase level. Bone scans are often helpful. In moderate-to-severe symptomatic disease, calcitonin limits the unregulated chaotic bone resorption and exerts highly specific and effective suppressive activity.
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PMID:Paget's disease: New treatment for an old disease. 13 63

The usual features of Paget's disease of bone, e.g. large head, deafness due to involvement of the 8th cranial nerve and bowing of the legs are all well described in text books of medicine. However, some features such as platybasia with neurological deficit, involvement of the 2nd, 5th and 7th cranial nerves, hydrocephalus, dementia, dysphasia, corrugation of the skull, bone pain (with or without associated osteo-arthrosis of hips and knees), heart failure, triradiate deformity of the pelvis have either had an inadequate or no description in books. These features are unusual, but it is important to recognise them, as the advances made since the introduction of the very promising new drugs in recent years have not only provided increasing insight into the disease, but also have improved management of patients with Paget's disease of bone.
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PMID:Some unusual features of Paget's disease of bone. 68 80

After reviewing the old ideas (peripheral syndrome and cardiac insufficiency at high outputs) and more recent ideas (arterial and intracardiac vascular calcification), the authors report their experiences based on the study of the histories of 150 patients suffering from Paget's disease followed for several years. First they emphasize the frequent latency of cardiac manifestations looked for systematically by means of the electrocardiogram particularly during evolutive stages of the disease. Electrocardiographic disturbances were observed 91 times and included 43 cases of conduction disorders and 48 of repolarization disorders; their distribution was studied as a function of various inherent parameters, of the patient (age and sex) and of the disease (its evolutive character and its degree of diffusion). On the basis of the anti-arythmic power of calcitonin found during experiments and obtained excellent results three times. Finally the place of cardiovascular manifestations in the pathogenic hypotheses and nosology of Paget's disease is discussed.
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PMID:[The cardiovascular manifestations of Paget's disease]. 110 62

The pathogenesis, clinical features, indications for therapy, and current pharamacologic management of Paget's disease are reviewed. Paget's disease is a bone disorder of unknown etiology primarily affecting the elderly. Overactive bone resorption leads to the accelerated formation of disorganized, weak bone. Pain and fractures are common clinical features. Neurologic, cardiovascular, metabolic, and neoplastic complications are also reported. Because most patients are asymptomatic, the disease is often detected during routine roentgenography or laboratory tests. Primary indications for pharmacologic intervention include bone pain, neural compression, immobilization hypercalcemia or hypercalciuria, cardiac failure, and orthopedic surgery. Recurrent or non-healing fractures and rapidly progressing complications are additional indications. Drugs used in the management of Paget's disease include calcitonin, etidronate disodium, and plicamycin. Although these agents are efficacious, each has disadvantages. Clinical resistance to animal calcitonins may develop, and the cost of therapy may be prohibitive. Etidronate may induce ostemalacia. The use of plicamycin is limited by potentially severe toxicities. Dichloromethylene and aminohydroxypropylidene are promising diphosphonate compounds but are still investigational In those patients who are unresponsive to single-agent regimens, combination therapy may prove effective. Although many patients with Paget's disease do not require pharmacologic therapy, calcitonin and etidronate are the agents of choice when it is indicated.
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PMID:Pharmacologic management of Paget's disease. 266 12

Osteitis deformans is a focal disease of the osteoclasts characterised by increased bone resorption subsequently followed by increased bone formation leading to abnormal bone. A viral etiology seems increasingly probable, but remains unproven. 5-30% of the patients present with symptoms such as pain, deformity and fracture. Hearing loss, nerve- or root-compression, arthrosis and hyperuricaemia may complicate the disease while malignant degeneration, hypercalcemia and high output cardiac failure are rare. The diagnosis is based on X-ray findings but biopsy may be necessary in selected cases. The extent of the disease is revealed by bone scintigraphy and the activity of the disease reflected by urine hydroxyproline excretion and serum alkaline phosphatase.
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PMID:[Paget's osteitis deformans. Epidemiology and clinical picture]. 292 39

The development of specific inhibitors of bone resorption has revolutionized the treatment of Paget's disease. The diphosphonates, the calcitonins, and mithramycin are capable of inducing marked suppression of disease activity for prolonged periods as judged by biochemical, kinetic, and histologic techniques. Whereas the effects of the calcitonins and mithramycin persist only for the duration of treatment, diphosphonate treatment consistently results in a reduction of disease activity for many months or even years after stopping treatment. The question arises whether the long-term control of the disease activity confers significant clinical advantages to the patient. Relief of bone pain, spinal neurologic syndromes, immobilization hypercalcemia, and high-output cardiac failure are related to the degree of biochemical control attained by treatment. New bone formed during treatment is lamellar and radiologic progression of disease is favorably modified. It is not yet known whether long-term treatment will decrease bone enlargement and deformity or reduce the risk of fracture.
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PMID:Long-term follow-up observations on treatment in Paget's disease of bone. 295 Oct 50

This paper describes the diverse complications of Paget's osteopathy. Lesions arising from changes in the bone per se, those due to secondary anatomical changes, and metabolic abnormalities, are discussed in relation to their presentation, frequency, and severity. Important complications involving the skeletal structures and nervous system are described. Sarcomatous change in the abnormal bone remains the most serious lesion. Some changes recognised for decades, such as high output heart failure, are very rare. Other less-well-recognised features, including hyperuricaemia, may be common enough to justify simple screening tests for their detection whenever Paget's disease presents clinically.
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PMID:Complications of Paget's disease of bone. 622 55

The authors illustrate a case report of Paget's disease in an exclusively cranio-facial localisation. Paget's disease is a dystrophy of an osteo-condensing type. Excess bone remodelling activity, due primarily to hyperclastic hyperactivity, leads to accelerated resorption and excess tissue formation. This marked alteration of the metabolism causes the hypertrophy of the bones involved, with zones of greater density and increased vascularisation. Its clinical manifestations include morphological deformations and painful symptoms in the areas affected. Complications may include pathological fractures, medullary or radicular compression, heart failure (rare), sarcomatous transformation (approximately 1% of cases). The case reported here describes a 70-year-old who was admitted to the out-patient clinic of the Odontostomatological Division of the Ospedale Mauriziano in Turin for a dental check up. The patient had had 6 teeth removed during the past 2 weeks in a private clinic. At the time of the control a mucous crater was present, also involving the underlying bone, at the level of the extracted 2.6 tooth. The patient had kept the teeth which had been removed some of which showed that the roots were surrounded by alveolar bone. A dental panoramic x-ray and cranial x-ray were performed using 3 projections and showed the remodelling of the cranial theca and upper jaw. The suspicion of Paget's disease was confirmed by hematochemical tests, bone scintigraphy with calcium phosphate and by the histologic examination of the alveolar bone fragments adhering to the root surface of the extracted teeth.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Paget's disease with an exclusively maxillary location. A case report]. 793 81

High cardiac output failure/state (HCOF) is regular feature of some illnesses e.g. thiamine deficiency, hyperthyroidism, severe anemia, Paget's disease or arteriovenous fistulae. HCOF in multiple myeloma is reported quite rarely. 31-year-old man was admitted because of fatigue, dyspnea and subfebrilities. Heart rate was 116/min, sinus rythm blood pressure 110/60 mmHg. Chest film showed cardiomegaly with sings of interstitial pulmonary edema, echocardiography mild dilatation of the left ventricle with hyperkinetic wall motion and small pericardial effusion. Hemoglobin was 104 g/l, leukocyte count 13.5 x 10(9)/l with 30% of plasmatic cells. Serum protein electrophoresis demonstrated a monoclonal gammapathy, X ray studies of the skelet multiple osteolytic lesions. Diagnosis of plasmocytic leukemia-form of multiple myeloma was established and chemotherapy (vincristine + adriamycine + dexamethason) was started. Patient cardiac status deteriorated. Cardiac catheterisation demonstrated mean righ atrial pressure of 25 mmHg, mean pulmonary artery pressure of 28 mmHg and pulmonary artery wedge pressure of 24 mmHg. Co was 20.0 l/min (C.I. 11.5 l/min/m2). In continuing of chemotherapy and symptomatic therapy for heart failure patients status gradually improved and complete remission of the myeloma and normalisation of cardiac parameters was achieved. Heart failure in multiple myeloma patients has been attributed to amyloidosis of myocardium, hyperviscosity syndrome, co-existing CAD or anthracycline toxicity. HCOF should be considered in patients with clinical evidence of heart failure and normal left ventricular function.
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PMID:[Hypercirculatory heart failure in a patient with plasmacytic leukemia]. 855 97

It has been suggested that rheumatological disorders are underdiagnosed in patients with medical problems and that this might be rectified by incorporating a standard brief screening examination as part of the routine assessment of all patients admitted to hospital with medical conditions. Therefore the GALS screening examination was used to assess the prevalence of rheumatic disease in 100 patients admitted with acute medical problems and in a further 100 in the rehabilitative phase of their disease. The nature of locomotor dysfunction in all patients with a positive result was defined by an independent review and then sensitivity and specificity of the screening test was calculated for rheumatic disease in both populations. The median age of the two populations were 63 and 78 years respectively, with more females in the rehabilitation group. The overall prevalence of a positive screening test was 53% in the acute and 94% in the chronic disease groups, although the false positive rate in the rehabilitation patients was 30% due to factors other than rheumatic disorders limiting locomotor function (mainly orthopaedic and neurological conditions). The diagnosis of a rheumatological disorder was made de novo in a significant minority (10%) of patients and was usually amenable to treatment. The commonest rheumatic disorder was osteoarthritis which accounted for 55% of all rheumatic disease, followed by inflammatory joint disease (16%), and osteoporosis (12%). In addition to osteoporosis, Paget's disease of bone and polymyalgia rheumatica were found more frequently in those patients undergoing rehabilitation than in those admitted with an acute medical problem. A number of clinically important associations between medical and rheumatic disorders were found, such as stroke disease with shoulder capsulitis and heart failure with gout. The sensitivity of the GALS screening test was extremely high (92% and 100%), while its specificity fell in the rehabilitation group from 83% to 17%. None the less, it is felt that this study indicates that the routine use of this test should be considered as part of the assessment of all hospitalised patients with medical problems, whether acute or chronic.
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PMID:What is the prevalence of rheumatic disorders in general medical inpatients? 1172 16


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