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Pediatrics General 3f63787d

Childhood osteopetrosis is characterized by – a) B/L frontal bossingb) Multiple # (fracture)c) Hepatosplenomegalyd) Cataracte) Mental retardation

A
bc
B
c
C
ac
D
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High-Yield Explanation
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.

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