A.Osteopetrosis
B.Osteogenesis imperfecta
C.Fluorosis
D.Achondroplasia
The correct answer is : A.Osteopetrosis
NOTE: Osteopetrosis(also known as Marble Bone Disease,Albers-Schonberg Disease) is a clinicopathological syndrome characterized by sclerosis and thickening of the bones which appear with increased radiographic density.
This results from the imbalance between bone formation and bone resorption.
There is dysfunction in osteoclast production or function.The exact mechanism is unknown.However,Carbonic anhydrase deficiency is noted in osteoclasts.This enzyme makes acidic environment through hydrogen ion pumping by osteoclasts and helps in dissociation of calcium hydroxyapatite from bone matrix.Hence,bone resortion falis while bone formation continues and results in increased bone formation.
Osteogenesis Tarda:Autosomal dominant variety,more common.Pathological fracture or cranial nerve compression due to encroachment in foramina.X-rays show increased density of all the bones: cortices are widened, leaving narrow medullary canals; sclerotic vertebral end-plates produce a striped appearance (‘football-jersey spine’); the skull is thickened and the base densely sclerotic.Treatment is required only if complications occur.
Osteopetrosis congenita:This rare, autosomal recessive form of osteopetrosis is present at birth and causes severe disability. Bone encroachment on marrow results in pancytopenia,haemolysis, anaemia and hepatosplenomegaly. Foraminal occlusion may cause optic or facial nerve palsy.Osteomyelitis following, for example, tooth extraction or internal fixation of a fracture is quite common.Repeated haemorrhage or infection usually leads to death in early childhood.Treatment, in recent years, has focused on methods of enhancing bone resorption and haematopoeisis,e.g. by transplanting marrow from normal donors and by long-term treatment with gamma-interferon.
B.Osteogenesis imperfecta
C.Fluorosis
D.Achondroplasia
The correct answer is : A.Osteopetrosis
NOTE: Osteopetrosis(also known as Marble Bone Disease,Albers-Schonberg Disease) is a clinicopathological syndrome characterized by sclerosis and thickening of the bones which appear with increased radiographic density.
This results from the imbalance between bone formation and bone resorption.
There is dysfunction in osteoclast production or function.The exact mechanism is unknown.However,Carbonic anhydrase deficiency is noted in osteoclasts.This enzyme makes acidic environment through hydrogen ion pumping by osteoclasts and helps in dissociation of calcium hydroxyapatite from bone matrix.Hence,bone resortion falis while bone formation continues and results in increased bone formation.
Osteogenesis Tarda:Autosomal dominant variety,more common.Pathological fracture or cranial nerve compression due to encroachment in foramina.X-rays show increased density of all the bones: cortices are widened, leaving narrow medullary canals; sclerotic vertebral end-plates produce a striped appearance (‘football-jersey spine’); the skull is thickened and the base densely sclerotic.Treatment is required only if complications occur.
Osteopetrosis congenita:This rare, autosomal recessive form of osteopetrosis is present at birth and causes severe disability. Bone encroachment on marrow results in pancytopenia,haemolysis, anaemia and hepatosplenomegaly. Foraminal occlusion may cause optic or facial nerve palsy.Osteomyelitis following, for example, tooth extraction or internal fixation of a fracture is quite common.Repeated haemorrhage or infection usually leads to death in early childhood.Treatment, in recent years, has focused on methods of enhancing bone resorption and haematopoeisis,e.g. by transplanting marrow from normal donors and by long-term treatment with gamma-interferon.
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| Osteopetrosis(Dense bone) |
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