[Value of in vitro study of osteoclast precursors in the case of severe infantile osteopetrosis treated by bone marrow graft].

Abstract

BACKGROUND:

Pathogenesis of osteopetrosis is still debated. Testing the ability of osteoclastic progenitors to support the proliferation of functional cells may be useful in understanding pathogenesis.

CASE REPORT AND METHODS:

A diagnosis of osteopetrosis was made in a girl 1 month-old, born to consanguuineous parents. Bone marrow transplantation was uneffective at the age of 3 months but a second engraftment was successful at 5 months. Unfortunately, the patient died from severe thrombocytopenia at the age of 8 months. Long-term cultures of mononucleated cells from the patient's blood were performed before and after the bone marrow transplantation, with or without growth factors such as vitamin D3, IL-6 and IL-1. Similar studies were made from the patient's marrow obtained after transplantation; all results were compared with those obtained after culturing control cells from cord blood umbilical.

RESULTS:

Production of osteoclastic cells was mild in peripheral blood cultures; it was important in bone marrow cultures in presence of growth factors.

CONCLUSION:

These results suggest that osteopetrosis in our patient resulted from an intrinsic defect in progenitors of osteoclasts.

• Authors: A Gourdin, A Demulder, A Ferster, P Fondu
• Journal: Archives de pediatrie : organe officiel de la Societe francaise de pediatrie (Arch Pediatr) Vol. 4 Issue 4 Pg. 335-8 (Apr 1997) ISSN: 0929-693X [Print] France
• Vernacular Title: Intérêt de l'étude in vitro des précurseurs ostéoclastiques dans un cas d'ostéopétrose infantile sévère traité par greffe médullaire.
• PMID: 9183405 (Publication Type: Case Reports, English Abstract, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Blood Cells (pathology)
• Bone Marrow (pathology)
• Bone Marrow Transplantation
• Cell Differentiation
• Cells, Cultured
• Female
• Humans
• In Vitro Techniques
• Infant
• Osteoclasts (pathology)
• Osteopetrosis (pathology, therapy)
• Postoperative Period