Record number :
2443629
Title of article :
A Case Report of Malignant Infantile Osteopetrosis
Author/Authors :
Usta, Merve Sisli Etfal Education and Research Hospital - Istanbul - Turkey , Gulec, Seda Geylani Sisli Etfal Education and Research Hospital - Istanbul - Turkey , Karaman, Serap Sisli Etfal Education and Research Hospital - Istanbul - Turkey , Erdem, Ela Sisli Etfal Education and Research Hospital - Istanbul - Turkey , Emral, Hicran Sisli Etfal Education and Research Hospital - Istanbul - Turkey , Urgancı, Nafiye Sisli Etfal Education and Research Hospital - Istanbul - Turkey
Pages :
4
From page :
421
To page :
424
Abstract :
Background Malignant infantile osteopetrosis (MIOP) 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. Case Presentation We report a two-month old male infant, diagnosed as MIOP while investigating the cause of hepatosplenomegaly. The patient was referred for stem cell transplantation. Conclusion Malignant infantile osteopetrosis should be kept in mind as a rare cause of hepatosplenomegaly and the patient should be referred for stem cell transplantation before neurologic or visual impairment develops.
Keywords :
Osteopetrosis , Infant , Hepatomegaly , Splenomegaly
Journal title :
Astroparticle Physics
Serial Year :
2012
Link To Document :
بازگشت