Non Glomerular Disease

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  • Cancer of the cervix, colon and bladder
  • Non Glomerular Disease

    1. 1. Non-Glomerular Diseases Clinical Medicine I Patrick Carter MPAS, PA-C February 25, 2011
    2. 2. Objectives <ul><li>Define tubulo-interstitial nephropathy </li></ul><ul><li>Differentiate between acute interstitial nephropathy and chronic interstitial nephropathy based upon: </li></ul><ul><ul><li>Etiology – main causes both drug and non-drug </li></ul></ul><ul><ul><li>Clinical features </li></ul></ul><ul><ul><li>Laboratory findings </li></ul></ul><ul><ul><li>Treatment </li></ul></ul><ul><ul><li>Prognosis </li></ul></ul>
    3. 3. Objectives <ul><li>Define polycystic kidney disease (PKD) </li></ul><ul><li>Review the prevalence of PKD and most common long term prognosis </li></ul><ul><li>Review the diagnosis of PKD including: </li></ul><ul><ul><li>Etiology </li></ul></ul><ul><ul><li>Clinical features </li></ul></ul><ul><ul><li>Laboratory findings and imaging findings </li></ul></ul><ul><ul><li>Use of gene therapy </li></ul></ul><ul><ul><li>Treatment </li></ul></ul><ul><ul><li>Prognosis </li></ul></ul><ul><li>Define medullary sponge kidney identifying presenting signs and symptoms, laboratory findings, and treatment </li></ul>
    4. 5. Tubulointerstitial Diseases <ul><li>May be acute or chronic </li></ul><ul><ul><li>Acute </li></ul></ul><ul><ul><ul><li>Most commonly associated with toxins and ischemia </li></ul></ul></ul><ul><ul><ul><li>Characterized by interstitial edema, infiltration with PMN’s, and patchy areas of tubular cell necrosis </li></ul></ul></ul><ul><ul><li>Chronic </li></ul></ul><ul><ul><ul><li>Interstitial fibrosis and more widespread tubular abnormalities – atrophy, luminal dilatation, and thickening of the tubule basement membrane </li></ul></ul></ul><ul><ul><ul><li>Mononuclear cell predominance </li></ul></ul></ul>
    5. 6. Acute Tubulointerstitial Disease <ul><li>Causes </li></ul><ul><ul><li>Drugs </li></ul></ul><ul><ul><ul><li>Antibiotics, NSAIDs, Diuretics, PPIs, H2 receptor blockers </li></ul></ul></ul><ul><ul><li>Infection </li></ul></ul><ul><ul><ul><li>Bacteria – strep, staph, salmonella </li></ul></ul></ul><ul><ul><ul><li>Viruses – EBV, CMV, HIV </li></ul></ul></ul><ul><ul><li>Ischemia </li></ul></ul><ul><ul><ul><li>Renal Artery Stenosis </li></ul></ul></ul>
    6. 7. Chronic Tubulointerstitial Disease <ul><li>Characterized by </li></ul><ul><ul><li>Decreased urinary concentrating ability </li></ul></ul><ul><ul><li>Reduced GFR </li></ul></ul><ul><ul><li>Small contracted kidneys </li></ul></ul><ul><li>Four main causes </li></ul><ul><ul><li>Obstructive uropathy – most common cause </li></ul></ul><ul><ul><li>Vesicoureteral reflux – 2 nd most common cause </li></ul></ul><ul><ul><li>Analgesics – NSAID’s, ASA, High Dose Tylenol </li></ul></ul><ul><ul><li>Heavy metals – Chronic Lead & Cadmium exposure </li></ul></ul>
    7. 8. Chronic Tubulointerstitial Disease <ul><li>Obstructive uropathy </li></ul><ul><ul><li>Prolonged obstruction of the urinary tract </li></ul></ul><ul><ul><ul><li>Urinary output alternates between Polyuria and Oliguria </li></ul></ul></ul><ul><ul><ul><li>Azotemia </li></ul></ul></ul><ul><ul><ul><li>Hypertension </li></ul></ul></ul><ul><ul><li>Major causes are prostatic disease, bilateral ureteral calculi, and cancer </li></ul></ul>} Due to increased renin-angiotensin production
    8. 9. Chronic Tubulointerstitial Disease <ul><li>Obstructive Uropathy </li></ul><ul><ul><li>Urinalysis - </li></ul></ul><ul><ul><ul><li>Often negative but can show </li></ul></ul></ul><ul><ul><ul><ul><li>Hematuria </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Pyuria </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Bacteriuria </li></ul></ul></ul></ul><ul><ul><li>CT scanning and MRI are best diagnostic tests </li></ul></ul><ul><ul><li>Treatment </li></ul></ul><ul><ul><ul><li>Treat underlying obstruction </li></ul></ul></ul>
    9. 10. Chronic Tubulointerstitial Disease <ul><li>Vesicoureteral reflux </li></ul><ul><ul><li>Second most common cause </li></ul></ul><ul><ul><li>Reflux is primarily a disorder of childhood </li></ul></ul><ul><ul><li>Due to incompetent vesicoureteral sphincter </li></ul></ul><ul><ul><li>Reflux of urine causes inflammatory response and fibrosis </li></ul></ul>
    10. 11. Chronic Tubulointerstitial Disease <ul><ul><li>Vesicoureteral reflux </li></ul></ul><ul><ul><ul><li>Kidney damage usually diagnosed in teens or 20’s </li></ul></ul></ul><ul><ul><ul><ul><li>Hypertension </li></ul></ul></ul></ul><ul><ul><ul><ul><li>Renal Insufficiency </li></ul></ul></ul></ul><ul><ul><ul><ul><li>History of Chronic UTI’s as child </li></ul></ul></ul></ul><ul><ul><ul><li>Diagnostic testing </li></ul></ul></ul><ul><ul><ul><ul><li>Cystography </li></ul></ul></ul></ul><ul><ul><ul><li>Treatment </li></ul></ul></ul><ul><ul><ul><ul><li>Surgical correction is necessary with severe reflux as this correlates with renal damage </li></ul></ul></ul></ul>
    11. 12. Chronic Tubulointerstitial Disease <ul><li>Analgesics </li></ul><ul><ul><li>Aspirin, NSAIDs (1 g/day for 3 years) </li></ul></ul><ul><ul><li>Leads to tubulointerstitial inflammation and papillary necrosis </li></ul></ul><ul><ul><li>Presents with hematuria, mild proteinuria, polyuria, anemia, and sterile pyuria </li></ul></ul><ul><ul><li>Diagnostic testing </li></ul></ul><ul><ul><ul><li>IVP to detect sloughed papillae (from papillary necrosis) </li></ul></ul></ul><ul><ul><ul><li>Cannot use in azotemia secondary to contrast </li></ul></ul></ul><ul><ul><li>Treatment </li></ul></ul><ul><ul><ul><li>Discontinue medication </li></ul></ul></ul>
    12. 13. Chronic Tubulointerstitial Disease <ul><li>Heavy metals </li></ul><ul><ul><li>Lead and cadmium </li></ul></ul><ul><ul><li>Welders (lead based paint) and moonshine drinkers (automobile radiators) </li></ul></ul><ul><ul><li>Lead accumulates in the proximal tubules </li></ul></ul><ul><ul><li>Commonly causes hypertension </li></ul></ul><ul><ul><li>Diagnostic testing </li></ul></ul><ul><ul><ul><li>Calcium disodium edetate (EDTA) chelation test – which is also used in treatment </li></ul></ul></ul>
    13. 14. Chronic Tubulointerstitial Disease <ul><li>Signs and symptoms </li></ul><ul><ul><li>Polyuria </li></ul></ul><ul><ul><ul><li>Due to inability to concentrate the urine </li></ul></ul></ul><ul><ul><li>Dehydration </li></ul></ul><ul><li>Laboratory findings </li></ul><ul><ul><li>Urinalysis is nonspecific </li></ul></ul><ul><ul><ul><li>Mild proteinuria </li></ul></ul></ul><ul><ul><li>Hyperkalemia </li></ul></ul><ul><ul><li>Hyperchloremic renal tubular acidosis </li></ul></ul><ul><ul><ul><li>Reduced ammonia production </li></ul></ul></ul><ul><ul><ul><li>Inability to acidify distal tubules </li></ul></ul></ul><ul><ul><ul><li>Proximal tubule bicarbonate wasting </li></ul></ul></ul>
    14. 15. Chronic Tubulointerstitial Disease <ul><li>Prognosis </li></ul><ul><ul><li>Depends on the disorder responsible </li></ul></ul><ul><ul><li>The degree of interstitial fibrosis can help predict recovery of renal function </li></ul></ul><ul><ul><li>Once there is loss of parenchyma, progression to ESRD can’t be prevented </li></ul></ul>
    15. 16. Polycystic Kidney Disease <ul><li>Key Features </li></ul><ul><ul><li>Common hereditary disease, affecting 500,000 individuals in United States </li></ul></ul><ul><ul><li>End-stage renal disease develops in 50% of patients by age 60 </li></ul></ul><ul><ul><li>10% of dialysis patients </li></ul></ul><ul><ul><li>Family history is positive in 75% </li></ul></ul>
    16. 17. Polycystic Kidney Disease <ul><li>Key Features </li></ul><ul><ul><li>Autosomal dominant </li></ul></ul><ul><ul><li>Two genes account for this disorder </li></ul></ul><ul><ul><ul><li>ADPKD1 on the short arm of chromosome 16 (85% of patients) </li></ul></ul></ul><ul><ul><ul><li>ADPKD2 on chromosome 4 </li></ul></ul></ul><ul><ul><li>Seen primarily in adults </li></ul></ul><ul><ul><li>Progressive expansion of numerous fluid-filled cysts results in massive enlargement of the kidneys </li></ul></ul>
    17. 18. Polycystic Kidney Disease <ul><li>Signs and symptoms </li></ul><ul><ul><li>Often asymptomatic until their 50s </li></ul></ul><ul><ul><li>Abdominal or flank pain </li></ul></ul><ul><ul><li>Abdominal mass </li></ul></ul><ul><ul><li>HTN (50%) </li></ul></ul><ul><ul><li>Fever (infection) </li></ul></ul><ul><ul><li>Hematuria </li></ul></ul><ul><ul><li>UTI </li></ul></ul>**Combination of HTN and an abdominal mass suggests the disease
    18. 19. Polycystic Kidney Disease <ul><li>Extrarenal manifestations </li></ul><ul><ul><li>Likely due to abnormality in collagen and extracellular matrix </li></ul></ul><ul><ul><li>SAH, cerebral hemorrhage - aneurysms in the circle of Willis in 10–15% </li></ul></ul><ul><ul><li>Aortic aneurysms </li></ul></ul><ul><ul><li>Aortic valve abnormalities – MVP, aortic regurg. </li></ul></ul><ul><ul><li>Hepatic cysts </li></ul></ul><ul><ul><li>Colonic diverticulae </li></ul></ul><ul><ul><li>Abdominal and inguinal hernias </li></ul></ul>
    19. 20. Polycystic Kidney Disease
    20. 21. Polycystic Kidney Disease <ul><li>Labs </li></ul><ul><ul><li>Urinalysis </li></ul></ul><ul><ul><ul><li>May be normal </li></ul></ul></ul><ul><ul><ul><li>Or show hematuria and mild proteinuria </li></ul></ul></ul><ul><ul><li>CBC </li></ul></ul><ul><ul><ul><li>Hgb and Hct often normal – erythropoietin produced by the cysts </li></ul></ul></ul>
    21. 22. Polycystic Kidney Disease <ul><li>Diagnosis </li></ul><ul><ul><li>Renal ultrasound </li></ul></ul><ul><ul><ul><li>Diagnostic criteria </li></ul></ul></ul><ul><ul><ul><ul><li>2 or > cysts in one kidney and at least 1 cyst in the contralateral kidney in < 30 years </li></ul></ul></ul></ul><ul><ul><ul><ul><li>2 or > cysts in each kidney age 30-59 </li></ul></ul></ul></ul><ul><ul><ul><ul><li>4 or > cysts in subjects >60 years (increased frequency of benign simple cysts) </li></ul></ul></ul></ul><ul><ul><li>CT scan or T2-weighted MRI is more sensitive in subjects <30 years of age </li></ul></ul><ul><ul><li>Cerebral arteriography screening if family history of aneurysms </li></ul></ul>
    22. 24. Polycystic Kidney Disease <ul><li>Treatment </li></ul><ul><ul><li>Largely supportive </li></ul></ul><ul><ul><li>HTN </li></ul></ul><ul><ul><ul><li>Aggressive Treatment (<130 / 85 is recommended) </li></ul></ul></ul><ul><ul><ul><li>Multidrug approach including agents to inhibit RAS </li></ul></ul></ul><ul><ul><li>Cyst rupture </li></ul></ul><ul><ul><ul><li>Bed rest </li></ul></ul></ul><ul><ul><ul><li>Analgesics, not NSAIDs </li></ul></ul></ul><ul><ul><li>Cyst pain </li></ul></ul><ul><ul><ul><li>Drainage by percutaneous aspiration </li></ul></ul></ul><ul><ul><ul><li>Sclerotherapy with alcohol </li></ul></ul></ul><ul><ul><li>Cyst infection </li></ul></ul><ul><ul><ul><li>Antibiotics: fluoroquinolones, trimethoprim-sulfamethoxazole </li></ul></ul></ul>
    23. 25. Polycystic Kidney Disease <ul><li>Prognosis </li></ul><ul><ul><li>Renal function declines progressively over the course of 10-20 years after diagnosis </li></ul></ul><ul><ul><li>Risk factors for progressive kidney disease include – younger age at diagnosis, AA race, male gender, HTN, ADPKD1 mutation </li></ul></ul><ul><ul><li>Can undergo kidney transplantation </li></ul></ul>
    24. 26. Medullary Sponge Kidney <ul><li>Relatively common and benign disorder </li></ul><ul><li>Autosomal dominant mutations in the MCKD1 or MCKD2 genes </li></ul><ul><li>Present at birth but not usually diagnosed until the 40s </li></ul><ul><li>Kidneys have irregular enlargement of the medullary and interpapillary collecting ducts </li></ul><ul><li>Medullary cysts causing a “Swiss cheese” appearance </li></ul>
    25. 27. Medullary Sponge Kidney
    26. 28. Medullary Sponge Kidney <ul><li>Signs/symptoms/clinical findings </li></ul><ul><ul><li>Polyuria - Decreased urinary concentrating ability </li></ul></ul><ul><ul><li>Gross or microscopic hematuria </li></ul></ul><ul><ul><li>Recurrent UTI’s </li></ul></ul><ul><ul><li>Nephrolithiasis </li></ul></ul><ul><li>Diagnosis </li></ul><ul><ul><li>IVP – striation in the papillary portions of the kidney </li></ul></ul><ul><ul><li>U/S or CT – hyperdense papillae with medullary cysts </li></ul></ul>
    27. 29. Medullary Sponge Kidney <ul><li>Treatment </li></ul><ul><ul><li>No known therapy </li></ul></ul><ul><ul><li>Adequate fluid intake to prevent stone formation </li></ul></ul>
    28. 30. Any Questions?

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