Cystic fibrosis and the kidney

Renal diseases in patients with cystic fibrosis F. Cachat Department of Pediatrics Pediatric nephrology unit Lausanne

CF: possible renal manifestations Birth Hypo- electrolytemia Pulmonary infections Pulmonary transplantation Kidney stones Nephrocalcinosis Drugs nephrotoxicity Other Glomerulonephritis Drugs Nephrotoxicity

CFTR expression in the kidney Cystic Fibrosis and the Kidney CFTR expression, variable during life, predominantly in the proximal and distal tubules

CFTR function Cystic fibrosis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

CFTR expression in the kidney ,[object Object],[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

CFTR and polycystic kidney disease ,[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

CFTR and polycystic kidney disease

CFTR and polycystic kidney disease ,[object Object],[object Object],Cystic Fibrosis and the Kidney

Hypoelectrolytemia ,[object Object],[object Object],[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Hypoelectrolytemia ,[object Object],[object Object],Cystic Fibrosis and the Kidney

Hypoelectrolytemia ,[object Object],[object Object]

Hypoelectrolytemia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Hypoelectrolytemia Cystic Fibrosis and the Kidney Important risk of sodium and water loss with concomitant hypokalemia and metabolic alkalosis secondary to CFTR mutation and worsened by particular environmental conditions

Kidney stones ,[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Kidney stones ,[object Object],[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Monohydrated calcium oxalate stones (whewellite) Monohydrated calcium oxalate crystals (whewellite)

Cystic Fibrosis and the Kidney Renal ultrasound demonstrating medullary nephrocalcinosis in a child with cystic fibrosis. From: Stephens SE et al. Cystic fibrosis and renal disease. Paediatr Resp Rev 2002;3:135-138

Kidney stones ,[object Object],[object Object],[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Repeated antibiotics administration Destruction of oxalobacter formigenes  enteric oxalate metabolism  free oxalate in the gut Hyperoxaluria / nephrocalcinosis Kidney stones Pancreatic insufficiency Decreased free calcium decreased calcium / oxalate complexion

Renal failure and CF ,[object Object],Cystic Fibrosis and the Kidney

Renal failure in patients with CF before lung transplantation ,[object Object],[object Object]

Aminoglycosides ,[object Object],[object Object],Cystic Fibrosis and the Kidney

[object Object],[object Object],[object Object],Al-Aloul M et al. Pediatr Pulmonol 2005;39:15-20

Transient Renal Failure Due to Simultaneous Ibuprofen and Aminoglycoside Therapy in Children with Cystic Fibrosis Kovesi TA 1998;338:65-66 Combination of intravenous aminoglycoside and Ibuprofen can cause acute renal insufficiency Ibuprofen and other NSAID interfere with the intrarenal production of PGE2 and prostacyclin, which cause renal vasodilation If used, ibuprofen (or other NSAID) should be discontinued during aminoglycosides therapy

Aminoglycosides Cystic Fibrosis and the Kidney ,[object Object],[object Object],[object Object],[object Object],[object Object]

Tubulo-interstitial nephritis ,[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Tubulo-interstitial nephritis Renal histology showing the lymphocytic infiltration of tubulo-interstitial nephritis and acute tubular necrosis. Stain: period acid-Schiff From: Stephens SE et al. Cystic fibrosis and renal disease. Paediatr Resp Rev 2002;3:135-138

Renal failure in patients with CF after lung transplantation

Important decrease of renal function in CF patients after lung transplantation

Numerous CF patients with lung transplant will need kidney replacement therapy/ transplantation Worse in CF patients than any other group Later age at Tx / antibiotic use / infections / diabetes mellitus

Glomerulonephritis and CF ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Urinary incontinence ,[object Object],[object Object],Cystic Fibrosis and the Kidney

Summary ,[object Object],[object Object],Cystic Fibrosis and the Kidney

Summary ,[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

Renal follow-up ,[object Object],[object Object],[object Object],[object Object],[object Object],Cystic Fibrosis and the Kidney

References: review ,[object Object]

References: CF and normal renal function ,[object Object],[object Object],[object Object],[object Object]

References: CF electrolytes / acid-base disorders ,[object Object],[object Object],[object Object],[object Object]

References: CF and kidney stones ,[object Object],[object Object],[object Object],[object Object]

References: CF and drug side effects ,[object Object],[object Object],[object Object],[object Object]

References: CF and chronic glomerulonephritis ,[object Object],[object Object],[object Object]

Cystic fibrosis and the kidney

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