Biology, Genetics & Bone Healing MCQs

Which of the following medical therapies is considered the most effective for the management of severe, recalcitrant bone pain associated with polyostotic fibrous dysplasia?

In cases of surgically inaccessible or highly morbid aneurysmal bone cysts (e.g., within the sacrum), which targeted systemic therapy has shown significant efficacy in shrinking the lesion and promoting ossification?

In the treatment of a recurrent aneurysmal bone cyst (ABC) of the pelvis that is deemed surgically unresectable due to excessive morbidity, which of the following medical therapies is most appropriate?

A 10-year-old child presents with severe bone pain secondary to polyostotic fibrous dysplasia. Nonsteroidal anti-inflammatory drugs have provided minimal relief. What is the most appropriate first-line pharmacologic therapy for managing this patient's bone pain?

A 40-year-old female presents with an atypical subtrochanteric femur fracture characterized by transverse orientation, lateral cortical thickening, and a medial spike. Which of the following medications is most directly associated with the pathogenesis of this fracture pattern?

A 28-year-old woman presents with knee pain. Radiographs show an eccentric, expansile, purely lytic lesion in the distal femoral epiphysis extending to the subchondral bone. Histology reveals mononuclear cells and multinucleated osteoclast-like giant cells. What targeted medical therapy has been FDA-approved for unresectable cases of this tumor?

A 5-year-old boy presents with an ability to touch his shoulders together anteriorly. He has a wide, flat skull, delayed fontanelle closure, and a wide pubic symphysis on radiographs. This condition is caused by a mutation in a gene crucial for the differentiation of which cell type?

A 10-year-old boy presents with progressive knee pain. Imaging reveals a subchondral lucency in the proximal tibial epiphysis. A biopsy demonstrates sheets of mononuclear cells with a 'coffee bean' nucleus and longitudinal grooving, alongside eosinophils. Which immunohistochemical marker is characteristically positive in this lesion?

A 72-year-old woman who has taken alendronate for 8 years sustains a low-energy transverse subtrochanteric femur fracture. Radiographs show lateral cortical thickening and a medial spike. Along with surgical fixation, what is the most appropriate long-term management of her bone health?

A 30-year-old woman presents with an expansile, lytic, epiphyseal lesion of the proximal tibia. Biopsy confirms Giant Cell Tumor (GCT) of bone. Denosumab is planned to aid in joint preservation. Denosumab acts primarily by inhibiting which of the following?

A 12-year-old boy presents with short stature, waddling gait, and early-onset osteoarthritis of the hips. Radiographs demonstrate irregular, delayed ossification of the capital femoral epiphyses and a double-layered patella. A mutation in which of the following genes is most likely responsible?

A 6-year-old girl is evaluated for delayed dental eruption and abnormal shoulder mobility. Physical examination reveals the ability to approximate her shoulders anteriorly to the midline. Cranial sutures are widened. A defect in which of the following transcription factors is responsible for this condition?

A 4-year-old child with blue sclerae, dentinogenesis imperfecta, and multiple long bone fractures is diagnosed with Osteogenesis Imperfecta (OI). Intravenous bisphosphonate therapy is initiated to reduce fracture burden. What is the primary cellular mechanism of action of this medication?

A 55-year-old female presents with severe pain, swelling, and stiffness of the hand 6 weeks after a distal radius fracture.

Which of the following prophylactic treatments has been shown to reduce the incidence of this complication if initiated acutely following the fracture?

A 10-year-old girl is able to bring her shoulders together anteriorly in the midline and exhibits delayed eruption of secondary teeth. What is the expected inheritance pattern and affected gene for this condition?

A child presents with frequent fractures, cranial nerve palsies, and hepatosplenomegaly. Radiographs show a generalized bone-in-bone appearance. The pathophysiology of this disease is primarily related to a defect in which of the following?

A 30-year-old female presents with knee pain. Radiographs show an eccentric, purely lytic epiphyseal lesion extending to the subchondral bone without a sclerotic rim. Biopsy confirms Giant Cell Tumor. Which medication targets the underlying molecular driver of the neoplastic cells?

A 32-year-old woman presents with knee pain. Radiographs reveal an eccentric, lytic lesion in the distal femur extending to the subchondral bone without a sclerotic margin. A biopsy confirms multinucleated giant cells. Which medication is an FDA-approved targeted therapy for unresectable or recurrent cases of this tumor?

A 65-year-old man with a known history of Paget's disease of bone sustains a subtrochanteric femur fracture after a minor fall. What is the most common underlying cause of increased bone turnover in this disease?