INFANTILE OSTEOPETROSIS - A case report
Journal: University Journal of Surgery and Surgical Specialities (Vol.5, No. 5)Publication Date: 2019-06-28
Authors : SARAVANARAJA B;
Page : 129-130
Keywords : Osteopetrosis; Compressive optic neuropathy; Malignant Infantile Osteopetrosis; SCT; Hepatosplenomegaly; Bone in bone appearance;
Abstract
Infantile malignant osteopetrosis presents early in life with extreme sclerosis of the skeleton and reduction of bone marrow spaces. Since there is a defect in the bone marrow, the disease can cause anemia, extramedullary hematopoiesis secondary to anemia leading to hepatosplenomegaly, cranial nerves compression and severe growth failure. This disorder is often lethal within the first decade of life because of secondary infections. Stem cell transplantation (SCT) remains the only curative therapy. We present a rare case of osteopetrosis in early infancy with classical clinical features and classical xray findings. Child is on regular followup withus and he is on supportive care.
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Last modified: 2019-07-01 18:34:51