Childhood osteopetrosis is characterized by – a) B/L frontal bossingb) Multiple # (fracture)c) Hepatosplenomegalyd) Cataracte) Mental retardation
Correct Answer: bc
Description: Osteopetrosis
Osteopetrosis is an autosomal recessive heredofamilial disorder in which partly calcified cartilaginous intercellular ground substance is not regularly reabsorbed & replaced by regular osteoid tissue & bone.
Manifested by :
Increased bone density masking the distinction between cortex & medulla, bone within bone appearance (Marble's bone).
Brittle bone & easily fracturability
Fracture healing satisfactory resulting in skeletal deformities.
Myelophthisic anemia
Hepatosplenomegaly & presence of immature leukocytes & normoblasts in the peripheral blood.
Growth retardation,Macrocephaly.
Thickening of cranial foramina & thus causing pressure on optic nerve & optic atrophy.
Deafness & blindness
Dental problems & osteomyelitis of the mandible.
Learning disorder but may have normal intelligence despite hearing & visual loss.
Category:
Pediatrics
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