NEPHROLOGY FOR STEP 3 By James K. Rustad, M.D. Copyright © 2009 All Rights Reserved. Outline Renal Failure (Post-Renal, Pre-renal and Intrarenal) Glomerulonephritis Nephrotic syndrome Acid-Base Disturbances Imbalances of Sodium Post-Renal Distended bladder on exam. Large volume diuresis after passing urinary catheter. Bilateral hydronephrosis on ultrasound. Post-void residual > 200 ml is suspicious. Black arrow = renal capsule Black arrowhead = sinus fat White arrow = dilated calyx White arrowhead = renal cortex Hydronephrosis Large hypoechoic area (black on U/S = no echoes) in center of kidney. Dilation extends into the parenchyma. The normal hyperechoic area in the center of the kidney (hilum) is replaced by a large hydronephrotic renal pelvis. This kidney has hydronephrosis due to obstruction of upper ureter. Treatment of Obstruction Lower tract: Foley cath. Upper tract: Ureteral stent or percutaneous nephrostomy. Pre-renal Hypotension. Hypovolemia (decreased renal perfusion). CHF: pump doesn’t work, can’t perfuse kidney. Constrictive pericarditis: heart cannot fill, can’t perfuse kidney. GI Bleed (digested blood is source of urea). Excess diuresis. Dehydrated patient may have high BUN (azotemia) due to lack of fluid volume to excrete waste products. Nothing wrong with kidney itself – will try to absorb sodium to maintain volume. Treat the underlying cause! Prerenal usually improves with IVF Pre-renal vs. Renal Pre-renal Renal BUN/Creatinine >20:1 <20:1 Urine sodium < 10 20 FENa <1 >1 Intra-Renal Acute Tubular Necrosis (ATN) Caused by hypoperfusion to the point of tubular cell death or from toxic injuries. Muddy Brown Casts ATN (continued) Contrast Nephropathy Typically begins immediately after study. Renal function recovers after 3-5 days. Prevent: Hydration, ½ NS 75 cc/hr 8-12 hr before and after study. Acetylcysteine 600 mg BID for 2 days if diabetes, CHF, CRI. Aminoglycoside Toxicity Begins at least 5 days after treatment. Rhabdomyolysis leading to ARF Labs: CPK elevated, Urine dipstick (urinalysis best initial test) shows blood but no RBC on microscopy. Urine myoglobin most accurate. Tx: IVF, IV sodium bicarbonate to alkalinize urine to solubilize myoglobin. Mannitol and diuresis decrease contact time of myoglobin with tubule. Clinical Situation Patient is brought to the emergency room after a seizure leading to prolonged immobility on sidewalk. Next step? A) Urinalysis B) Urine Myoglobin C) EKG D) CPK E) Phosphate level F) Creatinine Acute Rhabdomyolysis EKG most urgent step. Severe muscle necrosis leads to Hyperkalemia. Look for peaked T waves. Treat with immediate IV calcium gluconate, insulin, glucose. Order Potassium level (cellular destruction). Order Calcium level (hypocalcemia): damaged muscle may bind increased calcium. Hyperphosphatemia may lead to calcium binding with phosphate. Order chemistries to detect decreased serum bicarb. Acute Interstitial Nephritis The Pentad: Acute Renal Failure Fever Rash Arthralgia Peripheral blood eosinophilia Urine: WBC casts, eosinophil positive (Wright Stain) Causes: Penicillin, Sulfonamide, Rifampin, Allopurinol, Indinavir, 5-aminosalicylates, Proton Pump inhibitors. If NSAID induced: Fever, rash, eosinophilia, eosinophiluria usually absent. Treatment: Stop medication! Prednisone may be prescribed. Glomerulonephritis RBC Casts, Dysmorphic RBC Hematuria, proteinuria, HTN Causes: IgA Nephropathy Post-streptococcal GN Wegener’s granulomatosis Goodpasture Syndrome Endocarditis Lupus Nephritis IgA Nephropathy (Berger’s Disease) History of URI 1-2 days before presentation + Painless recurrent hematuria Recurrent hematuria following exertion Serum IgA increased in 50% of patients Normal complement Renal Biopsy to confirm Treatment: ACE inhibitor and ARB Consider adding Fish Oil Prednisone if tx-refractory or nephrotic syndrome/markedly proliferative disease. IgA Nephropathy Biopsy: Mesangial Hypercellularity IgA Nephropathy Immunofluorescence IgA mesangial deposits = characteristic and defining feature of the disease Deposits are exclusive of IgA in only ~26% of cases; usually accompanied by IgG (~37%) or IgM (~13%), and the three Igs may be present in ~25% of cases. However, IgA must be dominant or codominant Post-streptococcal GN URI 7-10 days before presentation “Cola or tea” colored urine Periorbital edema/HTN ASO titer, Low serum complement Treat infection/HTN Clinical Scenario Patient with sinusitis, rhinorrhea, bloody nasal discharge and cough, shortness of breath, hemoptysis. Hematuria, Proteinuria and Renal Failure. + for c-ANCA Most likely diagnosis? Wegener’s granulomatosis Confirm with biopsy of nasopharyngeal lesion if possible. Alternative: renal biopsy (crescentic necrotizing GN). Focal or diffuse necrotizing extracapillary GN is histological hallmark of ANCA-associated Vasculitis. Tx: Corticosteroid and Cyclophosphamide Good Pasture Syndrome Hemoptysis + Hematuria Proteinuria + Renal Failure Anti-GBM antibody! Tx: Plasmapheresis + Prednisone + Cyclophosphamide Lupus Nephritis Type I: Minimal Mesangial Type II: Mesangial Proliferative Type III: Focal Proliferative Type IV: Diffuse Proliferative Type V: Membranous Type VI: Advance Sclerosis Nephrotic Syndrome Not enough albumin (Hypoalbuminemia) Edema Proteinuria > 3.5 gm/24 hours Hyperlipidemia Nephrotic Syndrome Renal causes: Minimal Change Disease, Membranous Nephropathy, FSGS Systemic: SLE, Diabetes, Amyloidosis Minimal Change Disease Electron Microscopy Normal vs. MCD Normal Kidney MCD: The individual foot processes can no longer be made out- they have all just “melted” together into a single thin layer. Barrier in filtration process can no longer keep protein from being filtered out of the blood and into urine. Membranous Nephropathy Most common cause of Nephrotic syndrome in adults. Causes: idiopathic, carcinoma, Hep B, Hep C, SLE, Drugs: Penicillamine, Captopril, Gold Treatment: Prednisone Kidney Biopsy Review DIFFUSE When all or at least more than 80% of the glomeruli seen in the biopsy is affected. FOCAL Only certain proportion of the glomeruli seen in the biopsy involved. Kidney Biopsy Review GLOBAL When the entire globe of the glomerulus is affected . SEGMENTAL Only a segment of glomerulus involved. Focal Segmental Glomerular Sclerosis Most common cause of Nephrotic syndrome in black patients, obese patients, IVDA, heroin abuse, HIV. Acid-Base Disturbances Normal pH 7.4 = 40 nmol/L 24 X 40/24 H + = 24 X PCO2 (respiratory)/HCO3(metabolic) pH < 7.4 is acidotic Anion Gap (Na+) – (Cl- +HCO3-) MUDPILES Methanol Uremia DKA Paraldehyde/Propylene glycol INH/Iron Lactic acidosis Ethylene glycol Salicylates “My name is MUD!” Normal anion gap (8-12 mEq/L) Diarrhea Glue sniffing Renal tubular acidosis Hyperchloremia Metabolic Acidosis: Compensation For every 1 mEq HCO3- down, there is 1.3 mmHg PCO2 down Increased ventilation, blow off CO2 Metabolic Alkalosis Vomiting (urine chloride < 20) Diuretic use (except carbonic anhydrase inhibitors): Ucl>20 Antacids/milk-alkali syndrome Hyperaldosteronism Bicarbonate addition Loss of H+ from GI tract or kidney Loss of Chloride from GI tract or kidney Adaptation: 0.7 mm Hg of PCO2 up for every 1mEq/L up of HCO3Decrease ventilation! Respiratory Acidosis Primary disturbance? PCO2 Up Acute causes: Airway obstruction Status asthmaticus Alveolar defects (pulmonary edema, pneumonia) CNS depression, neuromuscular impairment Ventilatory restriction (flail chest with rib fractures) Chronic causes: COPD, abnormal chest wall mechanics. Compensation: Acute (for any PCO2 up go 1-3 mEq/L up in HCO3-) Chronic: For any 1 mm Hg up in PCO2 go up 0.4 mEq/L in HCO3-) Respiratory Alkalosis Primary disturbance? PCO2 down Cause: High altitude residence Pregnancy, Pulmonary disorder, CNS disease, Aspirin intoxification Hepatic failure, Sepsis Anxiety Hyperventilation High Carbon Dioxide Respiratory Acidosis Metabolic Alkalosis CO2 High High (compensation) pH <7.4 >7.4 Low Carbon Dioxide Respiratory Alkalosis Metabolic acidosis CO2 Low Low (compensatory) pH >7.4 <7.4 High Bicarbonate Metabolic Alkalosis Respiratory acidosis Bicarb High High (compensation) pH >7.4 <7.4 Low Bicarbonate Metabolic acidosis Respiratory alkalosis Bicarb Low Low (compensation) pH <7.4 >7.4 Clinical Scenario: Aspirin Overdose Causes two different primary disturbances: Respiratory Alkalosis and Metabolic Acidosis Look for co-existing tinnitus, hypoglycemia, vomiting, history of “swallowing several pills.” Alkalinize urine with Bicarbonate: speeds excretion. Imbalances of Sodium Hypernatremia Serum Na+ > 145 Causes: Diabetes insipidus, dehydration. Diabetes insipidus Lab: Urine osmolality less than serum. How do you differentiate between central and nephrogenic DI? Vasopressin challenge: Uosm increases in central, minimal change in nephrogenic. Central vs. Nephrogenic DI Central: Decreased production of vasopressin (trauma, neurosurgery, idiopathic). Treatment: intranasal DDAVP. Nephrogenic: renal tubules unresponsive to vasopressin (Lithium, Demeclocycline, hypokalemia, hypercalcemia). Treat the cause, thiazide or amiloride. Clinical Scenario 78 year old male from nursing home with altered mental status, serum sodium 160. BUN/Cr high and Uosm very high >800. Started DSW: after 10 hours the patient developed a seizure. What happened? Rapid correction of hypernatremia. In hypernatremic patient: slow correction 0.5 mEq/L per hour by free water or D5W (no more than 12 mEq/L over first 24 hours). Check serum sodium every 2 hours! Hyponatremia Hypovolemia Isovolemia Hypervolemia Diarrhea Vomiting Diuretics SIADH Hypothyroidism Cortisol insufficiency Polydipsia Beer Potomania CHF Nephrotic syndrome Cirrhosis Hyponatremia In True hyponatremia: Serum Osmolality should be low. Hyponatremia with normal plasma osmolality: Pseudohyponatremia due to hyperlipidemia or hyperproteinemia. Hyponatremia with elevated plasma osmolality: mannitol or hyperglycemia. For every 100 above 100 mg/dl of glucose > correct serum Na+ 1.6. Example: Plasma glucose 400 and Serum Na+ 130 > corrected will be 134.8 Treatment of Hyponatremia Asymptomatic: increase serum sodium 0.5 mEq/L per hour (10-12 mEq/L per day). Hypovolemia: Use Normal saline. Hypervolemia, SIADH: FLUID RESTRICTION! Symptomatic: Hypertonic saline, 1-2 mEq/L per hour first 3-4 hr then 0.5-1 mEq/L per hour. No more than 10-12 mEq/L per day in first 24 hours. Rapid Correction of Hyponatremia Central Pontine Myelinolysis or Osmotic Demyelination Syndrome (flaccid paralysis, dysarthria, dysphagia). Thank you for your attention!