关键词: 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

Mesh : Adult Child Child, Preschool Conservative Treatment / methods Female Humans Hyperoxaluria, Primary / diagnosis therapy Male Medication Adherence Mutation Transaminases / genetics Urinalysis / methods

来  源:   DOI:10.1016/j.nephro.2017.06.004   PDF(Sci-hub)

Abstract:
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.
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