In fact, many children with type C deformities also have acetabular dysplasia, a condition in which the acetabulum (hip socket) is shallow, abnormally shaped, and oriented outward. Type D — This is the most severe form of proximal femoral focal deficiency, in which most of the femur bone is absent and only a small irregular piece of bone above the distal femoral epiphysis (the end of the femur bone at the knee) is present. In the pelvis, no acetabulum (hip socket) is present; instead the pelvic wall is flat on the affected side. Treatment
Depending on the severity of the deformities, the treatment may include the amputation of the foot or part of the leg, lengthening of the femur, extension prosthesis, or custom shoe lifts. Amputation usually requires the use of prosthesis. Another alternative is a rotationplasty procedure, also known as Van Ness surgery. In this situation the foot and ankle are surgically removed, then attached to the femur. This creates a functional "knee joint". This allows the patient to be fit with a below knee prosthesis vs a traditional above knee prosthesis.In less severe cases, the use of an Ilizarov apparatus can be successful in conjunction with hip and knee surgeries (depending on the status of the femoral head/kneecap) to extend the femur length to normal ranges.
Tebentafusp, sold under the brand name Kimmtrak, is an anti-cancer medication used to treat uveal melanoma (eye cancer).The most common side effects include cytokine release syndrome, rash, pyrexia (fever), pruritus (itching), fatigue, nausea, chills, abdominal pain, edema, hypotension, dry skin, headache, and vomiting.Tebentafusp is a bispecific gp100 peptide-HLA-directed CD3 T cell engager. It was approved for medical use in the United States in January 2022. Medical uses
Tebentafusp is indicated for HLA-A*02:01-positive adults with unresectable or metastatic uveal melanoma. History
Efficacy was evaluated in IMCgp100-202 (NCT03070392), a randomized, open-label, multicenter trial of 378 participants with metastatic uveal melanoma. Participants were required to be HLA-A*02:01 genotype positive identified by a central assay. Participants were excluded if prior systemic therapy or localized liver-directed therapy were administered. Prior surgical resection of oligometastatic disease was permitted. Participants with clinically significant cardiac disease or symptomatic, untreated brain metastases were excluded.The U.S. Food and Drug Administration (FDA) granted Immunocores application for tebentafusp priority review, breakthrough therapy, and orphan drug designations. Society and culture
Legal status
On 24 February 2022, the Committee for Medicinal Products for Human Use (CHMP) of the European Medicines Agency (EMA) adopted a positive opinion, recommending the granting of a marketing authorization for the medicinal product Kimmtrak, intended for the treatment of uveal melanoma. The applicant for this medicinal product is Immunocore Ireland Limited. Tebentafusp was approved for medical use in the European Union in April 2022. References
External links
"Tebentafusp". Drug Information Portal. U.S. National Library of Medicine.