(Paget’s disease, osteitis deformans)

Paget’s disease of bone is a chronic disease of the adult skeleton, in which bone turnover is accelerated in circumscribed areas. The normal matrix is ??replaced by softer and enlarged bones. The disease can be asymptomatic or lead to gradually occurring bone pain or deformity. Diagnosis is made by radiography. The treatment includes symptomatic treatment and often medication, usually bisphosphonates.

About 1% of the adult US citizens (.. Editor’s note .: The prevalence in Western Europe is 1-2%) that are older than 40 years, have Paget’s disease with a male predominance of 3: 2. The prevalence of the disease increases with age. However, the overall prevalence appears to be declining. The disease is most common in Europe (excluding Scandinavia), Australia and New Zealand.

Paget’s disease of bone is a chronic disease of the adult skeleton, in which bone turnover is accelerated in circumscribed areas. The normal matrix is ??replaced by softer and enlarged bones. The disease can be asymptomatic or lead to gradually occurring bone pain or deformity. Diagnosis is made by radiography. The treatment includes symptomatic treatment and often medication, usually bisphosphonates. About 1% of the adult US citizens (.. Editor’s note .: The prevalence in Western Europe is 1-2%) that are older than 40 years, have Paget’s disease with a male predominance of 3: 2. The prevalence of the disease increases with age. However, the overall prevalence appears to be declining. The disease is most common in Europe (excluding Scandinavia), Australia and New Zealand. Etiology Several genetic abnormalities have been identified; many affect the receptor activator of “nuclear factor kappa-B” (RANK NFK B) is the driving force for the development and activity of osteoclasts. Mutations of Sequester- 1 gene linked to the ubiquitin-binding on chromosome 6 are present at about 10% of patients with Paget’s disease. The appearance of the affected bone in the electron suggests a viral infection. Although a viral cause has not been proven, it is believed that in genetically predisposed patients is a yet unidentified virus triggers an abnormal osteoclast activity. Pathophysiology It can be attacked at any bones. are most commonly affected in descending order pelvis, femur, skull, tibia, vertebrae, clavicle and humerus. The bone metabolism is elevated to the affected areas. Paget’s lesions are metabolically active and strong vascularized. The extremely active osteoclasts are often large and contain many nuclei. Osteoblastic repair is also overshoots and causes roughly woven, thickened lamellae and trabeculae. This abnormal structure weakens the bone, despite an increase and strong calcification. The complications too grown bone can nerves or other structures that pass through the narrow foramina compress. A spinal stenosis or spinal cord compression may occur. It may develop osteoarthritis in the adjacent on Paget’s bone joints. the increased bone formation and increased need for calcium to secondary hyperparathyroidism lead at about 10-15% of patients; If this requirement is not satisfied by an increase in calcium absorption occurs hypocalcaemia. Hypercalcemia (hypercalcemia) occurs occasionally in immobile patients. It also occurs in patients with Paget’s disease who develop secondary hyperparathyroidism. Large and numerous lesions can lead to fluid pressures of the heart. Highly vascularized bone can bleed excessively during orthopedic surgery. Symptoms and signs In most cases the disease for a long period without symptoms. If symptoms occur, they develop insidiously, with pain, stiffness, fatigue and bone deformation. Bone pain is sharp, deep and sometimes very strong, sometimes at night more pronounced. The pain may also be caused by nerve compression or osteoarthritis. If the skull is involved, headaches and hearing loss may occur. The findings further include a bitemporal and frontal skull enlargement, dilated cranial veins, nerval deafness in one or both ears, or dizziness, headache, pigment strip in the eye fundus, a kyphotic hull shortening with a primate-like appearance, a hobbling gait, an anterolateral bending of the thigh, leg, or humerus, often with warmth and tenderness. Deformities can be caused by the bending of the long bones or by a developing osteoarthritis. Pathological fractures may represent the first manifestation. Osteosarcoma occurs in less than 1% of cases, greatly increasing pain may indicate. Diagnostic X-rays serum levels of alkaline phosphatase, Ca and phosphate (PO 4) bone scan after detected diagnosis following findings substantiate the suspicion of Paget’s disease in patients: unexplained bone pain or deformity suspicious radiographic findings unexplained elevations of alkaline phosphatase in the serum of laboratory tests which were carried out for other reasons, especially when ?-glutamyl transpeptidase (GGT) is normal hypercalcemia, which develops during bed rest, especially in the elderly bone sarcoma in the elderly in all suspected Paget radiographs should be prepared and the serum AP, calcium and examined PO4. A confirmation by radiography is required to confirm the diagnosis. The following radiographic findings are characteristic: increased bone sclerosis abnormal architecture with coarse cortical trabecular and cortical thickening deformation bone expansion in the tibia or the femur can be found lateral microfractures. Typical laboratory results include increased serum AP (increased anabolic activity of bone), but mostly normal GGT and serum PO4 levels. The serum calcium level is usually normal, but may be (often temporarily) increased by increased bone synthesis due to immobilization or hyperparathyroidism, or due to a decrease. If the AP is not increased, or it is unclear whether the increase is made in the bone (i. E. When the GCD is increased in proportion to the AP), the bone-specific fraction can be determined. Tips and risks in older adults with elevated AP, but normal GGT level is to think of Paget’s disease of the bone. X-ray of the skull in Paget’s disease with permission of the publisher. From Maricic M., Ko M .: Atlas of Rheumatology. Edited by G. Hunder. Philadelphia, Current Medicine, 2005. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/xrays_skull_pagets_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual / professional / images / xrays_skull_pagets_high_de.jpg lang = en & thn = 0 ‘, title:’? radiograph of the skull with Paget’s disease ‘, description:’ u003Ca id = “v37892979 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eAuf this X-ray image can be seen a typical mosaic pattern of areas of increased sclerosis and increased radiolucency. This cotton-like spots are a result of a thickening of the cap u003c / p u003e u003c / div u003e. ‘Credits’ With permission of the publisher. From Maricic M.

Health Life Media Team

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