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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.
Insights
Type I primary hyperoxaluria causes bone fragility and fractures, even after combined liver-kidney transplantation. Preventive plating is recommended at the first sign of pain to address persistent orthopedic issues in these rare cases.
Area of Science:
- Nephrology
- Orthopedics
- Genetics
Background:
- Type I primary hyperoxaluria is a rare, autosomal recessive metabolic disorder.
- It results from a deficiency in a hepatic enzyme, leading to oxalate overproduction.
- Oxalate accumulation impacts multiple organs, notably bone.
Observation:
- The study analyzed orthopedic problems in children with Type I primary hyperoxaluria before and after combined liver and kidney transplantation.
- Two pediatric cases were presented and compared with existing literature.
- Orthopedic complications, especially femoral neck fractures, were noted to be common even post-transplantation.
Findings:
- Combined liver and kidney transplantation progressively improved osseous lesions and stromal appearance.
- However, transplantation did not prevent fractures, particularly at the femoral neck.
- Kidney transplant failure led to a persistent "congealed" clinical state with oxalate-rich bone.
Implications:
- Overproduction of calcium oxalate in Type I hyperoxaluria leads to systemic accumulation and bone fragility.
- Pathological fractures are a significant concern, necessitating early intervention.
- Preventive plating, possibly concurrent with transplantation, is suggested for patients experiencing initial pain to reduce fracture incidence.
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.