Bamberger-Marie syndrome

Hypertrophic pulmonary osteoarthropathy (Bamberger-Marie syndrome)Description:Expansion of the distal ends, or the entire shafts, of the long bones, sometimes with erosions of the articular cartilages and thickening and villous proliferation of the synovial membranes, and frequently clubbing of fingers; the disorder occurs in some chronic pulmonary diseases, in heart disease (most often congenital), and occasionally in other acute and chronic disorders.Also called as Hypertrophic pulmonary osteoarthropathy.ICD-9-CM: 731.2.Abbreviation: HPOA.Alternative Name: Hypertrophic pulmonary osteoarthropathy, hyperplastic osteoarthritis, pneumogenic osteoarthropathy, pulmonary osteoarthropathy,A syndrome characterised by clubbing of the fingers (Hippocratic fingers) and toes, periosteal new bone growth mainly in the long bones of the extremities, frequent evidence of joint involvement, and marked vasomotor disturbances involving hands and feet. It occurs in association with pulmonary disease such as bronchogenic carcinoma, occasional bronchiectasia, or lung abscesses. The syndrome is more common before middle life, and occurs more frequently in males.Hypertrophic Pulmonary Osteoarthropathy (HPOA) is described as bilateral, symmetric periosteal new bone formation, synovial effusions, and clubbing of the digits. The bone changes are seen in the distal extremities and begins as a low grade inflammatory process in the periosteum followed by new bone formation. Multiple layers of new bone are deposited which results in cortical thickening that can be seen on plain film. The diametaphysis of the tibia, fibula, radius, and ulna are usually involved. Other bony involvement includes the scapula which has been noted to be involved in two-thirds of the known cases, clavicles, ribs, and pelvic bones. The abnormalities are more obvious in the lower than upper extremities and again, in the bones distal to the knees and elbows rather than in the femurs and humeri.Hypertrophic Pulmonary Osteoarthropathy (HPOA) occurs in 5-10% of patients with intrathoracic malignancies (ie, bronchogenic carcinoma, pleural tumors, and less frequently lung metastasis). Many theories of HPOA have been developed. The latest consideration mentions the role of platelets and associated growth factors triggered by endothelial cells. Megakaryocytes and large platelet particles get fragmented in the passage through the diseased lung. These fragments interact with endothelial cells that trigger the release of growth factors causing proliferation of connective tissue and periosteum.There are two types of hypertrophic osteoarthropathy; primary and secondary. Secondary hypertrophic osteoarthropathy is more common than the primary and is seen in association with many disease conditions of the cardiovascular, hepatobiliary gastrointestinal and also in malignancies. Among the malignancies, carcinoma of the lung and breast are the commonest, which produces hypertrophic osteoarthropathy.HPOA most often affects the bones and joints of the wrists and ankles. Sometimes this shows up as changes on bone scans or x-rays. One of the most common symptoms of HPOA is a condition known as ` clubbing'. This means the nails, fingers and toes widen, curve and thicken. Clubbing of the fingers is a common symptom associated with lung and heart conditions. No one knows exactly why clubbing develops but it may be due to low oxygen levels in the bloodHypertrophic pulmonary osteoarthropathySymptoms:Hypertrophic pulmonary osteoarthropathy is a syndrome characterized by proliferative changes in the skin and skeleton. Proliferative periostitis of the long bones, oligopolysinovitis and digital clubbing are also common.HPOA can also affect the muscles and joints in a similar way to arthritis. The ankles and wrists become very swollen and inflamed causing a lot of pain and difficulty with movement. Treatment is usually with painkilling drugs such as non steroidal anti inflammatory drugs. Single treatments of radiotherapy can also sometimes help.Diagnosis:Bone scintigraphy with Tc-99m MDP [Methylene Diphosphonate] is a sensitive investigation in the detection of hypertrophic osteoarthopathy.Treatment Options:No drug effectively treats hypertrophic osteoarthropathy (HOA). Drugs such as NSAIDs may be used for symptomatic relief. Beta-blockers may be used for the treatment of hyperhidrosis of primary hypertrophic osteoarthropathy.Nonsteroidal anti-inflammatory drugs: These agents have analgesic, anti-inflammatory, and antipyretic activities. The main mechanism of action is inhibition of COX activity and prostaglandin synthesis. These agents may also have other mechanisms, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell membrane functions. NSAIDs such as ibuprofen, naproxen, indomethacin, piroxicam, diclofenac, and others are reasonable alternatives.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care. 


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