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Kidney: Fanconi and Fanconi-like syndromes

icanis

Synonym(s): Fanconi syndrome and proximal tubular acidosis


Introduction

  • Generalized dysfunction of the proximal renal tubules (at membrane or cellular level).
  • Cause: acquired or inherited.
  • Chronic renal failure can eventually occur, depending upon the underlying cause.
  • Severe renal tubular acidosis followed by papillary necrosis can cause acute death.
  • Signs: PD/PU, weight loss.
  • Treatment: replenish solutes, treat metabolic acidosis.
  • Prognosis: variable depending upon cause and stage of disease, guarded to poor for inherited or advanced disease.

Pathogenesis

Etiology

Predisposing factors

General
  • Breed.

Pathophysiology

  • The proximal tubular regulates urinary glucose, amino acids, phosphate, bicarbonate, potassium, sodium, calcium, magnesium, uric acid and organic acids.
  • Generalized proximal renal tubular dysfunction can result in a combination of reduced reabsorption of several solutes and subsequently increased urinary loss of glucose, amino acids, phosphate, bicarbonate, potassium, sodium, calcium, magnesium, uric acid, organic acids and reduced urine concentration (isosthenuria Urinalysis: specific gravity ; USG <1.018).
  • This in turn can result in reduced serum levels of potassium and phosphate and a hyperchloremic metabolic acidosis.
  • Metabolic acidosis can be self-limiting where the distal tubule continues fo function as acid (H+) can be excreted here, and as a result urine pH during acidosis generally is <6.0.
  • Dysfunction can progress to tubular atrophy and interstitial fibrosis with chronicity, resulting in renal failure.

Timecourse

  • Acquired disease can be reversible with removal of the inciting cause.
  • Cases can often be medically managed with stable disease for years.
  • Death can occur within months of diagnosis, especially where disease is advanced.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Referred papers

  • Recent references from PubMed and VetMedResource.
  • Langlois D K, Smedley R C et al (2013) Acquired proximal renal tubular dysfunction in 9 labrador retrievers with copper-associated hepatitis (2006-2012). JVIM 27 (3), 491-499 PubMed.
  • Thompson M F, Fleeman L M et al (2013) Acquired proximal renal tubulopathy in dogs exposed to a common dried chicken treat: retrospective study of 108 cases (2007-2009). Aust Vet J 91 (9), 368-373 PubMed.


Other sources of information

  • Kerl M E (2010) Renal Tubular diseases. In: Textbook of Veterinary Internal Medicine7th edn. Ettinger S J, Feldman E C (eds). St Louis, Missouri: Saunders Elsevier. pp 2062.
  • Brown S A (1989) Fanconi's syndrome - inherited and acquired. In: Current Veterinary Therapy X. Kirk R W, Bonagura J D (eds). Philadelphia: W B Saunders Co. pp 1163-1165.

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