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.
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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)
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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
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