Which of the following cells in his bones was most likely functionally deficient and replaced following transplantation?

A 2-year-old child has a history of multiple bone fractures with minor trauma. On examination he has hepatosplenomegaly and palsies involving cranial nerves II, VII, and VIII. Laboratory studies show pancytopenia. Radiographs reveal diffusely and symmetrically sclerotic bones with poorly formed metaphyses. Molecular analysis of his bone reveals a defect in production of carbonic anhydrase to solubilize hydroxyapatite crystal. He is treated with bone marrow stem cell transplantation.
1. Chondroblast
2. Chondrocyte
3. Osteoblast
4. Osteoclast
5. Osteocyte

Asked by Chapman, Last updated: Apr 05, 2024

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1 Answer

John Smith

Answered Sep 09, 2016

Osteoclast-(d) this is osteopetrosis (albers-schonberg disease), a rare bone disease resulting from mutations in genes thatregulate osteoclast activity and bone resorption. carbonic anhydrase generates the protons used by the h+-atpaseproton pump located on the osteoclast ruffled border. marrow is reduced within the sclerotic bone, with subsequentextramedullary hematopoiesis in spleen and liver. nerve compression in narrowed bony foramina leads to the palsies. theother cells listed are derived from mesenchymal stem cells, not hematopoietic stem cells. osteopetrosis is the opposite ofosteoporosis. remember that in osteoporosis the osteoclast formation and action are enhanced when osteoblasts produceless osteoprotegrin and m-csf, and increased rank and rankl interaction promote osteoclast differentiation andsurvival.bp7 757bp8 803pbd7 1281pbd8 12121214