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Clinical Pediatrics, Vol. 25, No. 8, 416-418 (1986)
DOI: 10.1177/000992288602500809

Pycnodysostosis with Visceral Manifestation and Rickets

B.R. Santhanakrishnan

Department of Pediatrics, Institute of Child Health and Hospital for Children, Egmore, Madras 600 008, India

S. Panneerselvam

Department of Pediatrics, Institute of Child Health and Hospital for Children, Egmore, Madras 600 008, India

S. Ramesh

Department of Pediatrics, Institute of Child Health and Hospital for Children, Egmore, Madras 600 008, India

M. Panchatcharam

Department of Pediatrics, Institute of Child Health and Hospital for Children, Egmore, Madras 600 008, India

Pycnodysostosis is a rare bone disease. Visceral manifestations associated with anemia and/or rickets have been reported in pycnodysostosis. Five children with typical findings of pycnodysostosis with hepatosplenomegaly, anemia with rickets, one with visceromegaly and anemia, and another with rickets alone are reported here. These findings strongly suggest that extramedullary erythropoiesis does occur in pycnodysostosis.


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