Osteopetrosis: A Report of Four Cases

  • Maria Abdulsalam Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Iskandar Wahidiyat Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Widhodho T. Karyomanggolo Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • L. A. Tamaela Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Keywords: osteopetrosis; thrombocytopenia, anemia, hepatosplenomegaly

Abstract

Four cases of osteopetrosis in infancy had been reported. All cases showed signs of anemia, thrombocytopenia and hepatosplenomegaly which at the beginning suggested the possibility of the diseases more commonly found in our country such as malaria, Cooley anemia, autoimmunne hemolytic anemia and malignancy. The authors then concluded that next to those diseases mentioned above the symptoms and signs of anemia, thrombocytopenia and hepatosplenomegaly especially in infancy should also lead to the possibility of the diagnosis of osteopetrosis. This latter is more obvious when it is accompanied by the difficulty in obtaining the bone marrow specimen.

References

1. ALTER, N.M.; PESEASE, M.C. and DE SANCTIS, A.G. : Albers-Schonberg's Disease; "Marble-Bones", Archs. Path. 11 : 509 (1931). Cited from Me Cune. D.J.; Bradley, C. : Osteopetrosis (Marble Bones) in an infant. Am. J. Dis. Child. 48 : 949 (1934).
2. BESSELMAN, D.M. : Splenectomy in the management of the anemia and thrombocytopenia of osteopetrosis (marble bone disease) J. Pediat. 69 : 455 (1966).
3. GAMSU, H.; LORBEK, J.; RENDLE-SHORT, J. : Haemolytic anemia in osteopetrosis; a report of two cases, Archs Dis. Childh. 36 : 494 (1961).
4. GRAHAM, C.B.; RUDHE, U.; EKLOF, 0. Osteopetrosis. Prog. pediat. Radiol. 4: 375 (1973).
5. HAMERSMA, H. Osteopetrosis (marble bone disease) at the Temporal. Laryngoscope 80 : 1518 (1970).
6. HAWKE, M.; JAHN, A.F.; BAILEY, D. : Osteopetrosis in the Temporal Bone. Archs Otolar. 107 : 278 (1981).
7. HILL, B.G.; CHARLTON, W.S.: Albers-Schonberg's disease. Med. J. Austr. 2 : 365 ( 1965)
8. HINKEL, C.L.; BEILER, D.D. : Osteopetrosis in adults. Am J. Roentg. 74 : 46 (1955).
9. NUSSEY, A.M. : Osteopetrosis. Archs Dis. Childh. 13 : 161 (193 8).
10. RAGAB, A.H.; DUCOS, R.; CHRIST, W.M.; DUCK, S.C. : Granulopoiesis in osteopetrosis. J. Pediat. 87:422 (1975).
11. RUBIN, P. : Dynamic Classification of Bone Displasias. (Year Book Med. Publ., Chicago 1964). Cited from Stelling, F.H. III : General Affections of the Skeletal System. Bone Displasias, Dystrophies and Dysostones. Pediat. Clins N. Am. : 14 : 359 (1967).
12.SCHWARTS, E. : Osteopetrosis; in Nathan, D.G.; Oski, F.A. : Hematology of Infancy and Childhood pp. 181 (Saunders, Philadelphia, London, Toronto 1974).
13. SJOLIN, S. : Studies on osteopetrosis. II. Investigations concerning the nature of the anemia. Acta paediat., U ppsala 4 8 : 5 29 ( 1959). Cited from Basselman, D.M. : Splenectomy in the management of the anaemia and thrombocytopenia of osteopetrosis (marble bone disease). J. Pediat. 69: 455 (1966).
14. STELLING, F.H. III : General Affections of the Skeletal System. Pediat. Clins N. Am. 14:355 (1967).
Published
2018-08-08
How to Cite
1.
Abdulsalam M, Wahidiyat I, Karyomanggolo W, Tamaela L. Osteopetrosis: A Report of Four Cases. PI [Internet]. 8Aug.2018 [cited 8May2024];24(9-10):211-4. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/1929
Received 2018-08-08
Published 2018-08-08