[Type 1 primary hyperoxaluria: From childhood to adult, how to manage adequately medical therapy compliance?]

Nephrol Ther. 2018 May;14(3):148-152. doi: 10.1016/j.nephro.2017.06.004. Epub 2017 Dec 6.
[Article in French]

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.

Keywords: 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.

Publication types

  • Case Reports

MeSH terms

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

Substances

  • Transaminases
  • Alanine-glyoxylate transaminase

Supplementary concepts

  • Primary hyperoxaluria type 1