Osteopetrosis (Marble Bone Disease) Quiz

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Questions and Answers

What is the characteristic radiographic feature of Osteopetrosis?

Decreased skeletal density
Presence of bone cysts
Increased skeletal density with a marble-like appearance (correct)
Widening of skull foramina

What is the age range when Cherubism typically presents?

5 to 10 years of age
1 to 2 years of age
10 to 15 years of age
2 to 5 years of age (correct)

What is the oral manifestation of Osteopetrosis?

Increased risk of dental caries
Absence of osteoclasts
Early exfoliation of teeth
Failure of tooth eruption (correct)

What is the complication of Osteopetrosis that affects the hematopoietic system?

All of the above (D) Signup and view all the answers

What is the differential diagnosis of Osteopetrosis?

All of the above (D) Signup and view all the answers

What is the primary reason for the blue sclera color in Osteogenesis Imperfecta Type I?

Reduced collagen content in the sclera allowing pigmented choroid to show through (D) Signup and view all the answers

Which of the following is a characteristic radiographic feature of Osteogenesis Imperfecta?

Bowing of long bones (B) Signup and view all the answers

What is the primary difference between Osteogenesis Imperfecta and Osteopetrosis?

Bone density (D) Signup and view all the answers

What is the outcome for most patients with Osteogenesis Imperfecta Type II?

Death before 4 weeks of age (A) Signup and view all the answers

What is the underlying cause of Osteopetrosis?

Failure of osteoclastic bone resorption (D) Signup and view all the answers

Which of the following congenital bone diseases is characterized by brittle bone that is prone to fracture?

Osteogenesis imperfecta (D) Signup and view all the answers

What is the primary cause of bone fragility in osteogenesis imperfecta?

Mutation in COL1A1 and COL1A2 genes (B) Signup and view all the answers

Which type of osteogenesis imperfecta is the most common and mildest form?

Type I (B) Signup and view all the answers

What is the characteristic feature of osteopetrosis?

Abnormal bone density (C) Signup and view all the answers

Which of the following is NOT a type of hereditary bone disease affecting endochondral bone?

Osteoporosis (C) Signup and view all the answers

What is the primary consequence of osteoporosis in the maxilla and/or mandible?

Enhanced bone fragility (C) Signup and view all the answers

Which congenital bone disorder is characterized by brittle bones that are prone to fracture?

Osteogenesis Imperfecta (C) Signup and view all the answers

What is the most significant factor in the prevention of osteoporosis?

Regular exercise (A) Signup and view all the answers

Which of the following is a characteristic of osteopetrosis?

Impaired bone remodeling (C) Signup and view all the answers

What is the primary focus of the lecture on osteoporosis and congenital bone disease?

Clinical and histopathological features of congenital bone disease (D) Signup and view all the answers

What is the primary genetic defect that leads to Cherubism?

Mutations in the RUNX2 gene (C) Signup and view all the answers

What is a characteristic oral manifestation of Cleidocranial Dysplasia?

All of the above (D) Signup and view all the answers

What is a common finding in radiographic examination of Cherubism patients?

Bilateral, multilocular radiolucencies (C) Signup and view all the answers

What is a characteristic feature of teeth in Cleidocranial Dysplasia patients?

Enamel hypoplasia (A) Signup and view all the answers

What is a common finding in the maxilla of Cleidocranial Dysplasia patients?

Narrow and V-shaped with high arched palate (D) Signup and view all the answers

What is the primary mechanism by which glucocorticoids contribute to bone loss?

Stimulation of osteoclastic activity (D) Signup and view all the answers

Which of the following is a consequence of mutation in COL1A1 and COL1A2 genes?

Deficiency in type 1 collagen (D) Signup and view all the answers

Heavy alcohol consumption can lead to bone loss and fracture due to which of the following mechanisms?

Increased risk of falling and poor nutrition (B) Signup and view all the answers

Which of the following medications is used to treat osteoporosis by inhibiting osteoclastic function and differentiation?

Bisphosphonates (A) Signup and view all the answers

Which of the following is a characteristic feature of osteoporosis?

Thin trabeculae disconnected from each other (B) Signup and view all the answers

What is the primary cause of achondroplasia?

Mutation in fibroblast growth factor 3 (FGFR3) on chromosome 4 (C) Signup and view all the answers

What is the characteristic feature of the axial skeleton in achondroplasia?

Normal development (A) Signup and view all the answers

What is the oral manifestation of achondroplasia?

Delayed dental development (B) Signup and view all the answers

What is the definition of osteoporosis?

A condition characterized by low bone mass (D) Signup and view all the answers

What is the consequence of osteoporosis?

Increased morbidity and mortality risks (C) Signup and view all the answers

What is the impact of achondroplasia on intelligence?

No impairment (A) Signup and view all the answers

What is the characteristic feature of the extremities in achondroplasia?

Short and thick (B) Signup and view all the answers

What is the consequence of delayed dental development in achondroplasia?

Delayed eruption of primary and permanent teeth (C) Signup and view all the answers

What is the characteristic feature of the maxilla in achondroplasia?

Hypoplasia (A) Signup and view all the answers

What is the type of malocclusion associated with achondroplasia?

Class III (C) Signup and view all the answers

Flashcards

Osteopetrosis radiographic feature?

Increased skeletal density with a marble-like appearance on radiographs.

Age of Cherubism presentation?

Typically presents between 2 to 5 years of age.