ORPTH ADONS DSE of OSSEOUS STRUCTUREs Flashcards
- Anomalies or malformations of individual bones or their pairs
is referred to as
(a) Dysplasia
(b) Dysostosis
(c) Dystrophy
(d) Enostosis
(b) Dysostosis refers only to defects or malformations of
individual or pairs of bones and does not refer to any
generalized disorder of the skeleton.
- Generalized abnormalities of cartilage or bone growth and
development is called as
(a) Osteochondrodysplasia
(b) Osteochondrometaplasia
(c) Osteoid dysplasia
(d) Chondrometaplasia
(a) This term signifies a generalized disorder of skeletal system
involving multiple bones at the time of presentation.
- Lobstein’s disease is another name of
(a) Osteogenesis imperfecta
(b) Dentinogenesis imperfecta
(c) Osteopetrosis
(d) Paget’s disease
(a) Osteogenesis imperfecta comprises a heterogeneous group of
heritable disorders characterized by impairment of collagen
maturation.
- Osteogenesis imperfecta results from an abnormality in _______
collagen.
(a) Type III
(b) Type II
(c) Type I
(d) Type IV
(c) Abnormal maturation of collagen results in bone with a thin
cortex, fine trabeculation, and diffuse osteoporosis
- In which of the following diseases is pale blue sclera not seen?
(a) Paget’s disease
(b) Marfan syndrome
(c) Ehlers-Danlos syndrome
(d) Pierre Robin anomalad
(d) This anomaly comprises of three skeletal components, i.e.
micrognathia, cleft palate and glossoptosis.
- Characteristic skeletal defects like arachnodactyly, dolichostenomelia and hyperextensibility of joints are features of
(a) Pierre Robin anomalad
(b) Ehlers-Danlos syndrome
(c) Cleidocranial dysostosis
(d) Marfan syndrome
. (d) This is a spectrum of disorders caused by a heritable genetic
defect of connective tissue that has an autosomal dominant
mode of transmission.
- Hypophosphatasia is a rare inherited metabolic disorder which
refers to a lack of
(a) Alkaline phosphatase
(b) Acid phosphatase
(c) Hydroxyapatite
(d) Calcium phosphate
(d) It is a disease of decreased tissue nonspecific alkaline
phosphatase TNSALP and defective mineralization. A
mutation in the ALPL gene, coding for TNSALP is believed
to be the cause.
- Failure of osteoclasts to resorb bone thereby resulting in
thickened sclerotic bones with reduced mechanical strength is
a characteristic feature of
(a) Osteogenesis imperfecta
(b) Osteopetrosis
(c) Paget’s disease
(d) Fibrous dysplasia
(b) Defective osteoclastic bone resorption, combined with
continued bone formation and endochondral ossification,
results in thickening of cortical bone and sclerosis of the
cancellous bone
- Hyperparathyroidism is most commonly associated with which
syndrome?
(a) von Recklinghausen’s disease
(b) Cleidocranial dysostosis
(c) Multiple endocrine neoplasia Type 1
(d) Fibrous dysplasia
(c) It is a syndrome of hypercalcemia caused by excessive
release of the parathyroid hormone. In 85% of cases primary
hyperparathyroidism results from an adenoma in a single
gland.
- The common site of involvement of polyostotic form of fibrous
dysplasia is
(a) Bones of skull and face
(b) Femur
(c) Ribs
(d) Spine
(a) Involvement of the mandible often results not only in
expansion of the lingual and buccal plates but also bulging of
the lower border. When the maxilla is involved, the lesional
tissue displaces the sinus floor superiorly and commonly
obliterates the maxillary sinus
- Monostotic form, polyostotic form, craniofacial form and
cherubism are disease patterns of which disease?
(a) Paget’s disease
(b) Fibrous dysplasia
(c) Cleidocranial dysostosis
(d) Brown’s tumor
(b) It is a developmental tumor-like condition characterized by
replacement of normal bone by an excessive proliferation of
cellular fibrous connective tissue intermixed with irregular
bony trabeculae.
- Large café-au-lait spots with irregular margins, precocious puberty and a “ground glass” appearance of affected bones
(a) Paget’s disease
(b) Pierre Robin anomalad
(c) Fibrous dysplasia
(d) Osteogenesis imperfecta
(c) Polyostotic fibrous dysplasia (affecting multiple bones)
along with café-au-lait spots is termed Jaffe-Lichtenstein
syndrome and if it occurs along with café-au-lait spots
and multiple endocrinopathies, then it is termed McCuneAlbright syndrome.
- All of the following except __________________ are histological
features found in fibrous dysplasia.
(a) Irregular, Chinese character-shaped bony trabeculae
(b) Osteoblastic rimming of bony trabeculae
(c) Larger than normal osteoblasts
(d) Collagen fibers of trabeculae extending into fibrous tissue
(b) Rimming by plump appositional osteoblasts is missing in
fibrous dysplasia as the bone trabeculae in this disease are
produced as a result of metaplasia.
- The most common malignancy arising in fibrous dysplasia is
(a) Fibrosarcoma
(b) Osteosarcoma
(c) Ewing’s sarcoma
(d) Multiple myeloma
(b) Osteosarcoma developing in fibrous dysplasia has been
reported in patients who received radiation therapy for
the treatment of this disease. Hence, radiotherapy is
contraindicated.
- McCune-Albright syndrome is also better known as
(a) Monostotic fibrous dysplasia
(b) Paget’s disease
(c) Polyostotic fibrous dysplasia
(d) Hyperparathyroidism
(c) When polyostotic fibrous dysplasia occurs along with café-
au-lait pigmentation and multiple endocrinopathies, then
it is termed McCune-Albright syndrome.