Clinical Approach to Proximal Renal Tubular Acidosis in Children

Gal Finer*, Daniel Landau

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

2 Scopus citations

Abstract

Proximal renal tubular acidosis (pRTA) is an inherited or acquired clinical syndrome in which there is a decreased bicarbonate reclamation in the proximal tubule resulting in normal anion gap hyperchloremic metabolic acidosis. In children, pRTA may be isolated but is often associated with a general proximal tubular dysfunction known as Fanconi syndrome which frequently heralds an underlying systemic disorder from which it arises. When accompanied by Fanconi syndrome, pRTA is characterized by additional renal wasting of phosphate, glucose, uric acid, and amino acids. The most common cause of inherited Fanconi syndrome in the pediatric age group is cystinosis, a disease with therapeutic implications. In this article, we summarize the clinical presentation and differential diagnosis of pRTA and Fanconi syndrome and provide a practical approach to their evaluation in children.

Original languageEnglish (US)
Pages (from-to)351-357
Number of pages7
JournalAdvances in Chronic Kidney Disease
Volume25
Issue number4
DOIs
StatePublished - Jul 2018

Keywords

  • Cystinosis
  • Dent disease
  • Fanconi syndrome
  • Lowe Syndrome
  • Proximal renal tubular acidosis

ASJC Scopus subject areas

  • Nephrology

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