Legg-Calve-Perthes Disease (LCPD)

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MedlinePlus Genetics: ⁴² Legg-Calvé-Perthes disease is a bone disorder that affects the hips. Usually, only one hip is involved, but in about 10 percent of cases, both hips are affected. Legg-Calvé-Perthes disease begins in childhood, typically between ages 4 and 8, and affects boys more frequently than girls.In this condition, the upper end of the thigh bone, known as the femoral head, breaks down. As a result, the femoral head is no longer round and does not move easily in the hip socket, which leads to hip pain, limping, and restricted leg movement. The bone eventually begins to heal itself through a normal process called bone remodeling, by which old bone is removed and new bone is created to replace it. This cycle of breakdown and healing can recur multiple times. Affected individuals are often shorter than their peers due to the bone abnormalities. Many people with Legg-Calvé-Perthes disease go on to develop a painful joint disorder called osteoarthritis in the hips at an early age.

MalaCards based summary: Legg-Calve-Perthes Disease, also known as perthes disease, is related to osteonecrosis and synovitis. An important gene associated with Legg-Calve-Perthes Disease is COL2A1 (Collagen Type II Alpha 1 Chain), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Defects of contact activation system (CAS) and kallikrein/kinin system (KKS). The drugs Anesthetics and Tocopherol have been mentioned in the context of this disorder. Affiliated tissues include hip joint, bone and bone marrow, and related phenotypes are delayed skeletal maturation and avascular necrosis

GARD: ¹⁹ Legg-Calve-Perthes disease (LCPD) occurs when blood supply to the ball of the thighbone in the hip (femoral head) is disrupted. Without an adequate blood supply, the bone cells die. Early symptoms may include limping; pain in the hip, thigh or knee; and reduced range of hip motion. Later in the disease course, there may be leg length discrepancy (one leg longer than the other) and wasting of the muscles around the hip. Some people with LCPD go on to develop degenerative arthritis in adulthood. The underlying cause of LCPD is often not known. Sometimes it results from a slipped capital femoral epiphysis, trauma, steroid use, sickle-cell crisis, toxic synovitis, or congenital hip dysplasia. It usually is not caused by genetic factors, but a few familial cases with autosomal dominant inheritance have been reported.

OMIM®: ⁵⁷ Legg-Calve-Perthes disease (LCPD) is characterized by loss of circulation to the femoral head, resulting in avascular necrosis in a growing child. Clinical pictures of the disease vary, depending on the phase of disease progression through ischemia, revascularization, fracture and collapse, and repair and remodeling of the bone. The disease occurs more frequently in boys, and most patients tend to be shorter than their peers. Both familial and isolated cases of LCPD have been reported (summary by Chen et al., 2004). (150600) (Updated 08-Dec-2022)

UniProtKB/Swiss-Prot: ⁷³ Characterized by loss of circulation to the femoral head, resulting in avascular necrosis in a growing child. Clinical pictures of the disease vary, depending on the phase of disease progression through ischemia, revascularization, fracture and collapse, and repair and remodeling of the bone.

Orphanet: ⁵⁸ A rare disorder characterized by uni- or bilateral avascular necrosis (AVN) of the femoral head in children.

Disease Ontology: ¹¹ An osteochondrosis that results in death and fracture located in hip joint.

Wikipedia: ⁷⁵ Legg-Calvé-Perthes disease (LCPD) is a childhood hip disorder initiated by a disruption of blood flow to... more...

Clinical features from OMIM®:

Search NIH Clinical Center for Legg-Calve-Perthes Disease