Nephrol Ther. 2017 Nov 29. pii: S1769-7255(17)30583-7. doi: 10.1016/j.nephro.2017.06.004. [Epub ahead of print]

Leflot M, Krzesinski JM, Collard L, Thomas A, Ghuysen MS.

We report the cases of three young patients suffering from type 1 primary hyperoxaluria, a metabolic genetic disorder characterized by intracellular accumulation of oxalate and which may result in end-stage renal disease with systemic impairment. A number of effective conservative therapeutic means are available for early management of affected children particularly when he is growing older. Despite the demonstrated efficacy of conservative therapy, compliance represents a major and daily challenge. Monitoring therapeutic compliance is thus an important task for physicians in charge of this disease. A better understanding of non-compliance causes is required to improve the follow-up of patients for whom treatment education must be a priority.

Hyperoxalurie primaire de type 1; Kidney and liver transplantation; Kidney stones; Lithiases rénales; Observance thérapeutique; Therapeutic compliance; Transplantation rénale et hépatique; Type 1 primary hyperoxaluria

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