short review of potential renal involvment in children with CF form a clinical point of view

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

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

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

7. CFTR and polycystic kidney disease

13. 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

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

17. 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

19. 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

24. 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

27. 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

28. Renal failure in patients with CF after lung transplantation

29. Important decrease of renal function in CF patients after lung transplantation

30. 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