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Orthopaedic

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Metabolic Bone Diseases

Question
11 out of 42
 

A 1 O-year-boy presents with predisposition to fractures and anaemia. X-ray shows increased density of the bone. The most likely diagnosis is (AIPG 2009)



A Osteogenesis imperfect

B Osteopetrosis

C Pyknodysostosis

D Myelofibrosis

Ans. B

Osteopetrosis

Osteopetrosis - (Marble-bone disease, Albers-Schonberg disease) This is a disorder characterized by dense but brittle bones (marble bones)

"Osteopetrosis is one of the several conditions which are characterized by thickening and increased radiographic density of the bones

Causes of generalized increased bone density

a. Myeloproliferative - Myelosclerosis

b. Metabolic - renal osteodystrophy

c. Poisoning - Fluorosis

d. Neoplastic - Osteoblastic (carcinoma prostate and breast) lymphoma,

e. Idiopathic - Paget's disease

X-ray shows - increased density of all the bones, cortices are widened, leaving narrow medullary canals, sclerotic vertebral endoplates produces a striped appearance ("rugger Jersey spine") the skull is thickened and the base densely sclerotic.

Treatment - transplanting marrow from normal donors, suggesting that the conditions is due to lack of marrow cells that control osteoclastic activity,

Metabolic Bone Diseases Flashcard List

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