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Case report


An infant with splenohepatomegaly: a rare cause.

Kathiravan KalyanasundaramPodhini JegadeesanSibi Chakravarthy MohanVinoth N Ponnurangam
Date of Submission: 31-Dec-1969, Date of Acceptance: 31-Dec-1969, Date of Web Publication: 30-Aug-2014.
Corresponding Author:
Corresponding Author

Vinoth N Ponnurangam,

Sri Ramachandra Medical College, Porur, Chennai - 600 116, Tamil Nadu, India.
E-mail: Vindoc1977@gmail.com

Corresponding Author:
Corresponding Author

Vinoth N Ponnurangam,

Sri Ramachandra Medical College, Porur, Chennai - 600 116, Tamil Nadu, India.
E-mail: Vindoc1977@gmail.com

DOI: 10.4103/2156-7514.139738 Facebook Twitter Google Linkedin

ABSTRACT


Osteopetrosis is a rare congenital disorder of bone resorption, caused by failure of osteoclasts to reabsorb immature bone. Malignant infantile osteopetrosis presents in early life with generalized osteosclerosis and decreased bone marrow spaces, resulting in anemia, splenohepatomegaly due to extramedullary hematopoiesis, cranial nerve compression, and growth failure. It is a fatal condition with death occurring within the first year of life. Bone marrow transplant remains the only curative treatment. We present a report of an infant with splenohepatomegaly, who was diagnosed with malignant infantile osteopetrosis.
Keywords: Bone Marrow Transplantation, Osteopetrosis, Osteosclerosis, Splenohepatomegaly

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