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Renal anomalies (VACTERL)

(50–80%)

Major anomalies (50.9%)

  • Renal agenesis
  • Renal dysplasia
  • Lobulated, fused and horseshoe kidneys

Minor anomalies (16.4%)

  • Polycystic kidneys and other cystic kidney diseases
  • Congenital obstructive defects of renal pelvis and malformations of the ureter

Why might these patients present to a GP as adults?

  1. Known patients with renal disease. Those in whom renal conditions were diagnosed in infancy/ childhood. These patients may remain under adult renal physician surveillance, but if not, renal function and blood pressure should be regularly monitored, particularly in light of recurrent UTIs.
  2. Undiagnosed patients with renal anomalies. Some patients may be diagnosed later on with renal anomalies, especially vesico-ureteric reflux, and this late diagnosis can increase risk of repeated UTIs, renal scarring and renal damage.
  3. Nephrolithiasis (kidney stones). The combination of renal and urological anomalies increases risk of kidney stones and in turn risk of renal damage.
  4. Progression of known kidney disease. VACTERL patients with known renal anomalies develop end-stage renal failure and require transplantation more frequently than those with renal anomalies without VACTERL.

Recommendation: Recurrent UTI, kidney stones and pyelonephritis need to be carefully investigated and monitored in VACTERL population to preserve renal function, and VACTERL patients with renal anomalies ideally should remain under monitoring of renal and/or urological adult specialists. (161,170–172)

References