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Legg–Calvé–Perthes disease

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Perthes' disease rarely occur after age 14 years (if diagnosed after 14 years of age, then it is usually old disease from early in childhood or avascular necrosis from an alternative cause). White northern Europeans appear to be affected more frequently than other ethnicities, though a paucity of reliable epidemiology exists in the Southern Hemisphere. Children of those with the disease themselves may have a very slightly increased risk, though it is unclear if this is because of a genetic predisposition, or a shared environmental factor. It is most commonly seen in persons aged three to twelve years, with a median of six years of age. The UK incidence rates show an intriguing pattern with low incidence rates in London, and a progressive increase in disease in more northerly areas (maximal in Scotland). Some evidence suggests, at least in developed countries, more socioeconomically deprived communities have a greater risk of disease (a similar trend to diseases such as adult heart disease), though the reason for this remains unknown. One possible explanation that has been considered is tobacco smoke exposure, though this is significantly confounded by the strong socioeconomic gradient common to both smoking and Perthes' disease.
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disease is a vascular restrictive condition of idiopathic nature. Symptoms like femoral head disfigurement, flattening, and collapse occur typically between ages four and ten, mostly male children of Caucasian descent. Children affected by LCP disease often display uneven gait and limited range of motion, and they experience mild to severe pain in the groin area. For example, a child may be six years old chronologically but may have grown only four years old in terms of bone maturity. The child may then engage in activities appropriate for a six-year-old child, but lacking the bone strength of an older child, these activities may lead to flattening or fracture of the hip joint. Genetics do not appear to be a determining factor, but a deficiency of blood factors with anticoagulant property used to disperse blood clots may lead to blockages in the vessels supplying the joint. A deficiency of
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rotation (with the knee flexed 90°, twisting the lower leg away from the center of the body). The range of motion is reduced, particularly in abduction and internal rotation, and the patient presents with a limp. Pain is usually mild. Atrophy of thigh muscles may occur from disuse and an inequality of leg length. In some cases, some activity can cause severe irritation or inflammation of the damaged area, including standing, walking, running, kneeling, or stooping repeatedly for an extended period. In cases exhibiting severe femoral osteonecrosis, pain is usually a chronic, throbbing sensation exacerbated by activity
265:). In some cases, pain is felt in the unaffected hip and leg, due to the children favoring their injured side and placing the majority of their weight on their "good" leg. It is predominantly a disease of boys (4:1 ratio). Perthes is generally diagnosed between 5 and 12 years of age, although it has been diagnosed as early as 18 months. Typically, the disease is only seen in one hip, but bilateral Perthes is seen in about 10% of children diagnosed. 522:
months, with a peak incidence around 7 months. The pathology of avascular necrosis followed by revascularization and bony remodeling of the femoral head in the dog certainly suggests a vascular etiology, though the cause of the condition is not completely understood. Hip pain is usually seen by the age of 6 to 8 months. The disease is bilateral in 12–16% of cases. X-rays are necessary to make the diagnosis and show increased opacity and focal
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is needed in the joint. As those affected age, problems in the knee and back can arise secondary to abnormal posture and stride adopted to protect the affected joint. The condition is also linked to arthritis of the hip, though this appears not to be an inevitable consequence. Hip replacements are relatively common as the already damaged hip experiences routine wear; this varies by individual, but generally is required any time after age 50.
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ten are at a very high risk of developing osteoarthritis and coxa magna. When an LCP disease diagnosis occurs after age eight, a better outcome results with surgery rather than nonoperative treatments. Shape of femoral head at the time when Legg-Calvé Perthes disease heals is the most important determinant of risk for degenerative arthritis; hence, the shape of femoral head and congruence of hip are most useful outcome measures.
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dysfunction, inflammation, nutrition, and altered circulatory hemodynamics. Risk factors are not limited to impaired and disproportionate growth, low birth weight, delayed skeletal maturity, short stature, systemic hormonal changes, and low economic index. Although no one has identified the cause of Perthes disease, a reduction in blood flow to the joint is known.
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physiotherapist to monitor progress. These exercises focus on improving and maintaining a full range of motion of the femur within the hip socket. Performing these exercises during the healing process is essential to ensure that the femur and hip socket have a perfectly smooth interface. This will minimize the long-term effects of the disease. Use of
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contact sports or games which impact the hip. For older children (onset of Perthes after age 6), the best treatment option remains unclear. Current treatment options for older children over age 8 include prolonged periods without weight bearing, osteotomy (femoral, pelvic, or shelf), and the hip distraction method using an
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hospital in England, Scotland, and Wales which treats Perthes' disease is collecting details of new cases. This study is supported by Steps Charity Worldwide. The study is nationally approved by the UK ethics committee and NHS research approval teams in England, Scotland, and Wales. The study is funded by the
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Perthes disease is self-limiting, but if the head of femur is left deformed, long-term problems can occur. Treatment is aimed at minimizing damage while the disease runs its course, not at 'curing' the disease. It is recommended not to use steroids or alcohol as these reduce oxygen in the blood which
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and implemented by an orthotist. Clinical results of the Scottish Rite orthosis have not been good according to some studies, and its use has gone out of favor. Many children, especially those with the onset of the disease before age 6, need no intervention at all and are simply asked to refrain from
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The disease is theorized to include the artery of the ligamentum teres femoris being constricted or even blocked too early, not allowing for time when the medial circumflex femoral artery takes over. The medial circumflex femoral artery is the principal source of blood supply to the femoral head. LCP
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can be affected. No sex predilection is seen in the dog as contrasted to humans, in whom an 80% male incidence of the disease is evident. Similar to children, however, the condition is usually unilateral, with only about 10–15% incidence of bilateral disease. The age of onset varies between 4 and 12
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Children younger than six have the best prognosis, since they have time for the dead bone to revascularize and remodel, with a good chance that the femoral head will recover and remain spherical after resolution of the disease. Children who have been diagnosed with Perthes' disease after the age of
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The condition is most commonly found in children between the ages of 4 and 10. Common symptoms include pain in the hip, knee, or ankle (since hip pathology can cause pain to be felt in a normal knee or ankle), or in the groin; this pain is exacerbated by hip or leg movement, especially internal hip
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Perthes' disease is one of the most common hip disorders in young children, occurring in roughly 5.5 of 100,000 children per year. The lifetime risk of a child developing the disease is about one per 1,200 individuals. Boys are affected about three to five times more often than girls. New cases of
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In the UK in 2016, a nationwide study began collecting details of every case of Perthes' disease as part of the British Orthopaedic Surgery Surveillance Study. This aims to get core information about the disease, prior to starting extensive clinical trials about the best forms of treatment. Every
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may be used. Overnight traction may be used in lieu of walking devices or in combination. These devices internally rotate the femoral head and abduct the leg(s) at 45°. Orthoses can start as proximal as the lumbar spine, and extend the length of the limbs to the floor. Most functional bracing is
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or MRI may be useful in making the diagnosis in those cases where X-rays are inconclusive. Usually, plain radiographic changes are delayed six weeks or more from clinical onset, so bone scintigraphy and MRI are done for early diagnosis. MRI results are more accurate, i.e. 97–99% against 88–93% in
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The goals of treatment are to decrease pain, reduce the loss of hip motion, and prevent or minimize permanent femoral head deformity so that the risk of developing a severe degenerative arthritis as an adult can be reduced. Assessment by a pediatric orthopaedic surgeon is recommended to evaluate
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LCP disease is an avascular necrosis of the femoral head in small-breed dogs, usually those weighing up to 25 lbs. LCP disease was first described in veterinary literature by Tutt in 1935. Tutt described the disease as almost exactly what Waldenstromin (1909) described in humans. Small breeds,
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Legg believed the cause was impairment of blood supply to the femoral epiphysis, Calvé believed rickets, and Perthes deduced an infection possibly causing degenerative arthritis leads to LCP disease. Currently, a number of factors have been implicated, including heredity, trauma, endocrine
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is highly recommended, as it allows exercise of the hip muscles with full range of motion while reducing the stress to a minimum. Cycling is another good option as it also keeps stress to a minimum. Physiotherapy generally involves a series of daily exercises, with weekly meetings with a
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The condition is most commonly found in children between the ages of 4 and 8, but it can occur in children between the ages of 2 and 15. It can produce a permanent deformity of the femoral head, which increases the risk of developing
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1910: The disease was recognized as being unrelated to tuberculosis by three physicians working independently. Legg–Calvé–Perthes is named after these three doctors: Arthur Legg (1874–1939), Jacques Calvé (1875–1954), and
530:, but conservative treatment (rest, exercise restriction, and pain medication) may be effective in a limited number of cases (less than 25%, according to some studies). In dogs, the prognosis is excellent with surgery. 348:
which only affects the hip, although other forms of osteochondritis can affect elbows, knees, ankles, and feet. Bilateral Perthes, which means both hips are affected, should always be investigated thoroughly to rule out
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Johannesen J, Briody J, McQuade M, Little DG, Cowell CT, Munns CF (November 2009). "Systemic effects of zoledronic acid in children with traumatic femoral head avascular necrosis and Legg-Calve-Perthes disease".
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While running and high-impact sports are not recommended during treatment for Perthes disease, children can remain active through a variety of other activities that limit mechanical stress on the hip joint.
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and a weakening of the femoral head. The bone loss leads to some degree of collapse and deformity of the femoral head and sometimes secondary changes to the shape of the hip socket.
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Bahmanyar S, Montgomery SM, Weiss RJ, Ekbom A (August 2008). "Maternal smoking during pregnancy, other prenatal and perinatal factors, and the risk of Legg-Calvé-Perthes disease".
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plain radiography. If MRI or bone scans are necessary, a positive diagnosis relies upon patchy areas of vascularity to the capital femoral epiphysis (the developing femoral head).
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Perry DC, Bruce CE, Pope D, Dangerfield P, Platt MJ, Hall AJ (December 2012). "Perthes' disease of the hip: socioeconomic inequalities and the urban environment".
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Perthes disease is a childhood hip disorder initiated by a disruption of blood flow to the head of the femur. Due to the lack of blood flow, the bone dies (
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Laine JC, Martin BD, Novotny SA, Kelly DM (August 2018). "Role of Advanced Imaging in the Diagnosis and Management of Active Legg-Calvé-Perthes Disease".
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Chou DT, Ramachandran M (2016). "Chapter 7: Legg-Calvé-Perthes' disease: Historical note". In Aresti NA, Ramachandran M, Paterson JM, Barry M (eds.).
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Seufert CR, McGrory BJ (October 2015). "Treatment of Arthritis Associated With Legg-Calve-Perthes Disease With Modular Total Hip Arthroplasty".
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Vosmaer A, Pereira RR, Koenderman JS, Rosendaal FR, Cannegieter SC (January 2010). "Coagulation abnormalities in Legg-Calvé-Perthes disease".
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Wainwright AM, Catterall A (2010). "Capter 27: Legg–Calvé–Perthes disease: Its name". In Benson M, Fixsen J, MacNicol M, Parsch K (eds.).
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Treatment has historically centered on removing mechanical pressure from the joint until the disease has run its course. Options include
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Balasa VV, Gruppo RA, Glueck CJ, Wang P, Roy DR, Wall EJ, et al. (December 2004). "Legg-Calve-Perthes disease and thrombophilia".
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of the hip may suggest and/or verify the diagnosis. X-rays usually demonstrate a flattened, and later fragmented, femoral head. A
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Over time, healing occurs by new blood vessels infiltrating the dead bone and removing the necrotic bone which leads to a loss of
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in the head of the femur, and later in the disease, collapse and fracture of the neck of the femur. The recommended treatment is
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achieved using a waist belt and thigh cuffs derived from the Scottish-Rite orthosis. These devices are typically prescribed by a
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Kealey WD, Moore AJ, Cook S, Cosgrove AP (March 2000). "Deprivation, urbanisation and Perthes' disease in Northern Ireland".
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which affects only the hip. Bilateral Perthes, which means both hips are affected, should always be investigated to rule out
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Perry DC, Bruce CE, Pope D, Dangerfield P, Platt MJ, Hall AJ (December 2011). "Perthes' disease: deprivation and decline".
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such as zoledronate or ibandronate is currently being investigated, but definite recommendations are not yet available.
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is unknown.Perthes can produce a permanent deformity of the femoral head, which increases the risk of developing
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which relieves the hip from carrying the body's weight. This allows room for the top of the femur to regrow.
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Kim HK, Herring JA (July 2011). "Pathophysiology, classifications, and natural history of Perthes disease".
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Kim HK (April 2012). "Pathophysiology and new strategies for the treatment of Legg-Calvé-Perthes disease".
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Barker DJ, Dixon E, Taylor JF (November 1978). "Perthes' disease of the hip in three regions of England".
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Pain in the hip, knee, ankle (hip pathology can refer pain to a normal knee or ankle), or groin.
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1909: Henning Waldenström (1877–1972) described the disease, attributing it to tuberculosis.
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risks and treatment options. Younger children have a better prognosis than older children.
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Perry DC, Machin DM, Pope D, Bruce CE, Dangerfield P, Platt MJ, Hall AJ (February 2012).
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The first signs are complaints of soreness from the child, which are often dismissed as
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X ray of deformity by Legg–Calvé–Perthes disease of the right hip (left side of X-ray)
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Joseph B (July 2011). "Prognostic factors and outcome measures in Perthes disease".
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Artery of the ligmentum teres femoris being constricted or even blocked too early
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Demko J, McLaughlin R (September 2005). "Developmental orthopedic disease".
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Wenger DR, Ward WT, Herring JA (June 1991). "Legg-Calvé-Perthes disease".
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Perry DC, Bruce CE, Pope D, Dangerfield P, Platt MJ, Hall AJ (May 2012).
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Osteochondrosis that results in death and fracture located in hip joint
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Hulth A, Norberg I, Olsson SE (July 1962). "Coxa plana in the dog".
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Tutt JF (1935). "Tuberculosis of the hip joint in a cairn terrier".
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Hall AJ, Barker DJ (March 1989). "Perthes' disease in yorkshire".
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Radiograph of a person with Legg–Calvé–Perthes disease
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Springer. p. 105. 1560:10.1007/s11999-011-2180-8 1090:10.1007/s11999-011-2240-0 1025:10.1016/j.ocl.2011.03.004 990:10.1016/J.OCL.2011.04.007 200: 192: 182: 174: 166: 156: 144: 68: 63: 54: 40: 35: 2477:Osgood–Schlatter disease 2383:Relapsing polychondritis 2255:Osteonecrosis of the jaw 2082:Osteitis fibrosa cystica 1745:Who's who in orthopedics 1714:Who's who in orthopedics 1681:Who's who in orthopedics 1618:Who's who in orthopedics 1316:Arthritis and Rheumatism 947:10.5435/JAAOS-D-16-00856 903:10.5435/JAAOS-D-13-00173 268: 2193:Paget's disease of bone 2144:Vertebral osteomyelitis 491: 320:of the capital femoral 2043:Bone and joint disease 736:10.1542/peds.2008-0307 437: 2523:Scheuermann's disease 2399:Slipping rib syndrome 2221:Hajdu–Cheney syndrome 511:Jack Russell Terriers 435: 281:) and stops growing. 2316:Aneurysmal bone cyst 2272:Nonossifying fibroma 1061:. New York: Praeger. 863:10.2106/JBJS.J.01834 825:10.2106/JBJS.I.00157 49:Legg–Perthes disease 1195:Step-Up to Medicine 479:Research directions 332:of the head of the 2721:Skeletal disorders 2664:External resources 2548:Kienböck's disease 2304:Skeletal fluorosis 2250:Avascular necrosis 1975:(31–32): 517–523. 1285:10.1093/aje/kwr293 1046:http://www.nih.gov 503:Yorkshire Terriers 438: 279:avascular necrosis 249:Signs and symptoms 223:avascular necrosis 2731:Syndromes in dogs 2698: 2697: 2576: 2575: 2572: 2571: 2568: 2567: 2412: 2411: 2355: 2354: 2287:Fibrous dysplasia 2152: 2151: 1931:(5): 1111–35, v. 1602:978-1-4471-6767-9 1472:(12): 1053–1057. 1359:(12): 1124–1128. 1329:10.1002/art.34316 1231:(12): 2642–2647. 1209:978-0-7817-7153-5 1165:(10): 1743–1746. 208: 207: 184:Diagnostic method 30:Medical condition 16:(Redirected from 2738: 2580: 2579: 2538: 2514: 2482:Blount's disease 2455: 2448: 2447: 2421: 2420: 2366: 2365: 2231:Gorham's disease 2198:Hypophosphatasia 2105: 2075: 2065: 2064: 2056: 2055: 2036: 2029: 2022: 2013: 2012: 2002: 1992: 1949: 1948: 1920: 1911: 1910: 1892: 1886: 1885: 1857: 1851: 1850: 1848: 1847: 1829: 1823: 1821: 1809: 1803: 1802: 1790: 1784: 1783: 1781: 1779: 1773:www.boss.surgery 1765: 1759: 1758: 1740: 1734: 1733: 1717: 1707: 1701: 1700: 1684: 1674: 1668: 1667: 1651: 1641: 1632: 1631: 1613: 1607: 1606: 1588: 1582: 1581: 1571: 1554:(9): 2369–2375. 1539: 1533: 1532: 1504: 1498: 1497: 1461: 1455: 1454: 1426: 1420: 1419: 1391: 1385: 1384: 1348: 1342: 1341: 1331: 1322:(5): 1673–1679. 1307: 1298: 1297: 1287: 1263: 1257: 1256: 1220: 1214: 1213: 1189: 1183: 1182: 1154: 1148: 1147: 1118: 1112: 1111: 1101: 1084:(9): 2462–2475. 1069: 1063: 1062: 1054: 1048: 1043: 1037: 1036: 1008: 1002: 1001: 984:(3): 285–95, v. 973: 967: 966: 930: 924: 923: 905: 881: 875: 874: 846: 837: 836: 808: 802: 801: 765: 756: 755: 730:(2): e459–e464. 719: 708: 707: 689: 683: 682: 672: 662: 638: 629: 618: 612: 611: 594:(4): e144–e149. 588:Pediatric Annals 583: 577: 576: 548: 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941:(15): 526–536. 931: 927: 882: 878: 847: 840: 809: 805: 776:(11): 676–686. 766: 759: 720: 711: 704: 690: 686: 639: 632: 619: 615: 584: 580: 549: 545: 540: 494: 481: 456: 447: 430: 375: 359: 346:osteochondritis 303: 271: 251: 239:osteochondritis 140: 113: 93: 79: 75: 45:Perthes disease 31: 28: 23: 22: 15: 12: 11: 5: 2744: 2734: 2733: 2728: 2723: 2718: 2713: 2696: 2695: 2692: 2691: 2680: 2668: 2667: 2665: 2661: 2660: 2657: 2656: 2645: 2634: 2623: 2608: 2592: 2587: 2586: 2584: 2583:Classification 2574: 2573: 2570: 2569: 2566: 2565: 2563: 2562: 2561: 2560: 2558:Panner disease 2552: 2551: 2550: 2541: 2539: 2531: 2530: 2528: 2527: 2526: 2525: 2517: 2515: 2505: 2504: 2502: 2501: 2500: 2499: 2494: 2492:Köhler disease 2486: 2485: 2484: 2479: 2469: 2468: 2467: 2458: 2456: 2445: 2439: 2438: 2436: 2435: 2429: 2427: 2418: 2414: 2413: 2410: 2409: 2407: 2406: 2401: 2395: 2393: 2389: 2388: 2386: 2385: 2380: 2374: 2372: 2363: 2357: 2356: 2353: 2352: 2350: 2349: 2344: 2343: 2342: 2340:Melorheostosis 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347: 343: 339: 335: 331: 327: 323: 319: 318:osteonecrosis 315: 310: 308: 298: 296: 292: 286: 282: 280: 276: 275:osteonecrosis 266: 264: 263:referred pain 260: 259:growing pains 255: 246: 244: 240: 236: 230: 228: 224: 220: 219:osteonecrosis 216: 212: 203: 199: 195: 191: 187: 185: 181: 177: 173: 169: 165: 161: 159: 155: 152: 149: 147: 143: 134: 74: 73: 71: 69:Pronunciation 67: 62: 58: 53: 50: 46: 43: 39: 34: 19: 2682: 2671: 2647: 2636: 2625: 2610: 2595: 2535: 2509: 2464: 2452: 2323:Hyperostosis 2308: 2188:Osteomalacia 2171:Osteoporosis 2166:Bone density 2129:Sesamoiditis 2099: 2069: 2060:Inflammation 1972: 1968: 1928: 1924: 1896: 1890: 1865: 1861: 1855: 1844:. 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2183:Osteopenia 2122:Involucrum 2117:Sequestrum 1846:2011-12-04 724:Pediatrics 653:(1): 125. 538:References 519:Dachshunds 316:avascular 314:idiopathic 2689:radio/387 2684:eMedicine 2310:bone cyst 2158:Metabolic 2101:infection 2071:endocrine 1381:206853890 627:eMedicine 428:Prognosis 400:physician 395:orthotics 373:Treatment 366:bone scan 357:Diagnosis 338:hip joint 322:epiphysis 307:bone mass 301:Pathology 295:protein S 291:protein C 227:bone mass 201:Frequency 196:Orthotics 193:Treatment 146:Specialty 2417:Combined 2176:Juvenile 1999:19730720 1945:16129135 1882:14036446 1578:22090357 1529:10755420 1494:30496559 1486:23104772 1373:22080458 1338:22143958 1294:22223709 1253:30054597 1245:15590848 1179:25979188 1144:19446052 1108:22270467 1033:21742142 998:21742140 963:49417062 955:29939867 920:38168231 912:25667404 871:22488623 833:20048104 798:42235121 790:21041802 752:12645569 744:18625663 679:35292045 608:27064472 413:Swimming 383:traction 158:Symptoms 2643:D007873 1990:2735835 1795:Vet Rec 1569:3830089 1451:2925740 1099:3830104 670:8922924 573:2045406 454:History 336:in the 324:of the 2678:001264 2632:150600 2554:elbow 2544:wrist 2226:Ainhum 1997:  1987:  1943:  1903:  1880:  1820:: 487. 1801:: 428. 1751:  1726:  1693:  1660:  1624:  1599:  1576:  1566:  1527:  1492:  1484:  1449:  1416:711792 1414:  1379:  1371:  1336:  1292:  1251:  1243:  1206:  1177:  1142:  1106:  1096:  1031:  996:  961:  953:  918:  910:  869:  831:  796:  788:  750:  742:  700:  677:  667:  606:  571:  517:, and 387:braces 362:X-rays 175:Causes 137: 2621:732.1 2606:M91.1 2511:spine 2488:foot 2472:tibia 2443:Child 2392:Other 2361:Joint 2240:Other 1490:S2CID 1377:S2CID 1249:S2CID 959:S2CID 916:S2CID 794:S2CID 748:S2CID 524:lysis 334:femur 269:Cause 188:X-Ray 2654:9891 2638:MeSH 2627:OMIM 2616:9-CM 2536:arm: 2461:hip 2453:leg: 2051:Bone 1995:PMID 1941:PMID 1901:ISBN 1878:PMID 1866:44-A 1780:2016 1749:ISBN 1724:ISBN 1691:ISBN 1658:ISBN 1622:ISBN 1597:ISBN 1574:PMID 1525:PMID 1482:PMID 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Index

Legg–Calvé–Perthes syndrome

/ˈlɛɡkælˈvˈpɜːrtz/
Specialty
Orthopedics
Symptoms
Diagnostic method
osteonecrosis
avascular necrosis
bone mass
osteoarthritis
osteochondritis
multiple epiphyseal dysplasia
growing pains
referred pain
osteonecrosis
avascular necrosis
protein C
protein S
bone mass
idiopathic
osteonecrosis
epiphysis
femoral head
blood supply
femur
hip joint
osteoarthritis
osteochondritis
multiple epiphyseal dysplasia

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