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[Iterative fractures in type I primary hyperoxaluria. Report of 2 cases]
1Service de Chirurgie Orthopédique et Traumatologie H.I.A. Ste Anne Bd Ste Anne, Toulon.
Purpose Of The Study:
Type I primary hyperoxaluria is a rare autosomal recessive disease linked to a deficit in an hepatic enzyme. The purpose of this study was to analyze orthopedics problems caused by type I primary hyperoxaluria before and after liver and kidney transplantation.
Material And Methods:
Two cases of children carrying this type I primary hyperoxaluria followed up after liver kidney transplantation are presented and compared to last publications.
Results:
Combined transplantation progressively corrected osseous lesions and aspect of the stroma. However it did not provide protection against fractures particularly for femoral neck fractures.
Discussion:
In type I hyperoxaluria overproduction of calcium oxalate causes its accumulation in the whole organism and particularly in bone. Osseous fragility favors pathological fractures. Only combined liverkidney transplantation can save and cure these children. Frequency of this fracture after transplantation indicates preventive plating at first pain, possibly at the same time as transplantation. Kidney transplant failure puts the patient in a "congealed" clinical state where the bone is very rich in oxalate and where the hemodialysis does not eliminate oxalate salts.
Conclusion:
Type I primary hyperoxaluria is a very rare disease. Fractures are very common even after liver and kidney transplantation and especialy femoral neck fractures. We think that preventive plating must be done at first pain. We do not have any explanation for bony weakness after liver-kidney transplantation.