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Nephrocalcinosis

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Nephrocalcinosis

  1. 1. NEPHROCALCINOSIS<br />DM SEMINAR<br />Dr. Vishal Golay<br />23/02/2011<br />
  2. 2. TOPIC OVERVIEW<br />Types of Nephrocalcinosis<br />Causes<br />Pathogenesis<br />Diagnostic work up<br />Treatment<br />Vishal Golay's<br />
  3. 3. INTRODUCTION<br />The true incidence of nephrocalcinosis is difficult to establish due to the wide range of etiologies.<br />Data available from case collections<br />Mortensen and Emmett-91 cases (1954)<br /> J Urol 71:398–406<br />Monserrat et al -77 cases (1979)<br />Wrong and Feest-375 cases (1976)<br /> Advanced medicine no 12. Pitman Medical, London, pp 394–206<br />Vishal Golay's<br />
  4. 4. INTRODUCTION<br />The term means an increase in the calcium content (generalized) of the kidney parenchyma.<br />Three forms:<br />Chemical Nephrocalcinosis<br />Microscopic Nephrocalcinosis<br />Macroscopic Nephrocalcinosis<br />Vishal Golay's<br />
  5. 5. Hypercalcemic Nephropathy<br />Vishal Golay's<br />
  6. 6. Hypercalcemic Nephropathy<br />Renal vasocontriction<br />Loss of concentrating capacity and resistance to vasopressin<br />Alkalosis/acidosis<br />K and Mg loss<br />Hypertension<br />Vishal Golay's<br />
  7. 7. Microscopic Nephrocalcinosis<br />Precipitation of Ca as phosphate or oxalate<br />Midway in spectrum b/w Hypercalcemia and macroscopic nephrocalcinosis.<br />Healthy kidneys contain Ca on autopsy.<br />Many times diagnosis is made biochemically<br />Vishal Golay's<br />
  8. 8. Vishal Golay's<br />
  9. 9. Macroscopic Nephrocalcinosis<br />The calcium deposits are visualized by imaging techniques.<br />Conventional X-ray, USG, CT and MRI can be used.<br />Can affect the cortex as well as the medulla depending on the etiology<br />Vishal Golay's<br />
  10. 10. Etiology<br />Cortical Nephrocalcinosis (2.4% of all cases)<br />Chronic Glomerulonephritis<br />Acute Cortical Necrosis<br />Chronic Pyelonephritis<br />Benign Nodular subcapsular type<br />Post transplant<br />Post traumatic<br />Oxalosis<br />Vishal Golay's<br />
  11. 11. Etiology<br />MedullaryNephrocalcinosis (97.6% of all cases)<br />Conditions causing hypercalcemia<br />Idiopathic Hypercalciuria<br />Oxalosis<br />dRTA<br />MSK<br />Renal papillary necrosis<br />Dent’s disease<br />Other rare causes<br />Vishal Golay's<br />
  12. 12. Pathogenesis<br />There are two types of microscopic crystal deposition-<br />One taking place within the tubular lumen (intratubularnephrocalcinosis), and <br />the other in the interstitium(interstitial nephrocalcinosis).<br />Vishal Golay's<br />Nephrol Dial Transplant (2009) 24: 2030–2035<br />
  13. 13. Intratubular nephrocalcinosis<br />Two main pathogenetic processes are involved<br />supersaturation<br />Crystal formation<br />failure of anti-crystal<br /> forming controls.<br />epithelial crystal adhesion<br />Crystal retention<br />tubular crystal obstruction<br />Vishal Golay's<br />
  14. 14. Intratubular nephrocalcinosis<br />Consequences:<br />Crystal obstruction cause damage due to the decrease in no. of functioning nephronsacutely and secondarily due to the chronic changes caused by it.<br />It can also lead to nephrolithiasis<br />Crystal adhesion cause slow damage, hampers redifferentiation/regeneration of functioning tubules and also can form nidus for growth of other crystals<br />Vishal Golay's<br />
  15. 15. Interstitial Nephrocalcinosis<br />Mechanisms of formation:<br />Transcytosis<br />Exocytosis<br />Exotubulosis<br />De novo formation Randall’s plaque<br />Vishal Golay's<br />
  16. 16. Vishal Golay's<br />Mechanisms involved in renal crystal handling and the development of nephrocalcinosis<br />
  17. 17. Some important causes of nephrocalcinosis<br />Vishal Golay's<br />
  18. 18. Hypercalcemia<br />Primary hyperparathyroidism is the most important cause (in adults) according to all the series.<br />Medullary location.<br />More closely related to the duration of hypercalcemia rather than the severity.<br />Degree of renal failure does not correlate with nephrocalcinosis.<br />Vishal Golay's<br />
  19. 19. Distal RTA<br />Nephrocalinosis is an important manifestation of dRTA although any condition causing NC can lead to dRTA.<br />Factors leading to stone formation in dRTA:<br />Hypercalciuria<br />Hypocitraturia<br />Alkaline urinary pH<br />Predominant crystal deposited is Calcium PO4<br />Vishal Golay's<br />
  20. 20. Medullary Sponge Kiney<br />Ectatic dilatation of the distal collecting tubules confined to the renal pyramids<br />Not exactly a true NC as the calcium deposition lies in the dilated tubules.<br />70% of the concretions is Calcium PO4<br />Relatively benign condition.<br />Diagnosis by radiology<br />Vishal Golay's<br />
  21. 21.
  22. 22. Clinical implications of NC<br />Features of the underlying primary condition.<br />Renal stone formation.<br />UTI<br />Polyuria and thirst (decreased concentrating capacity).<br />Renal Failure.<br />Hypertension is not a usual feature.<br />Vishal Golay's<br />
  23. 23. Clinical implications<br />Sterile pyuria (uniform finding).<br />Erythrocytosis.<br />Proteinuria is also not marked(except in Dent’s disease and Fanconi syndrome).<br />Impairment in urinary acidification (makes the diagnosis of dRTA difficult).<br />Vishal Golay's<br />
  24. 24. Natural History<br />Nephrocalcinosis generally persists for life with a few exceptions.<br />Barring a few exceptions the majority of the causes are incurable.<br />Many of the conditions can lead to development of renal failure.<br />Vishal Golay's<br />
  25. 25. Natural History<br />Probability of development of ESRD in various diseases causing NC:<br />Best:<br />1. Idiopathic hypercalciuria<br /> 2. MSK<br /> 3. dRTA<br /> 4. Hypercalcemic conditions<br /> 5. Papillary Necrosis<br /> 6. Dent’s Disease<br /> 7. Hypomagnesemia-hypercalcemia syndrome<br />Worst:<br /> Primary Hyperoxaluria type 1<br />Vishal Golay's<br />
  26. 26. Evaluation<br />Proper history and examination<br />Laboratory investigations:<br />RFT, Ca profile<br />Urinalysis<br />Urine c/s<br />24 hour timed urine examination<br />iPTH, TSH<br />Urinary Magnesium Levels<br />Vishal Golay's<br />
  27. 27. Evaluation (Radiological Investigations)<br />Overview:<br />Forms the basis of diagnosis.<br />May detect asymptomatic cases.<br />CT scan is the most sensitive and specific test but carries radiation risk.<br />USG has inter-observer variation.<br />Conventional radiography cannot detect NC until attenuation exceeds 100HU<br />Vishal Golay's<br />
  28. 28. X-Ray KUB<br />Requires attenuation values above 100HU<br /><2mm deposits are rarely picked up.<br />the spatial resolution of the recording technique, and contrast factors also influence the detection rate.<br />Useful as a screening tool<br />Vishal Golay's<br />
  29. 29. X-Ray KUB<br />Cortical Nephrocalcinosis:<br />Single cortical, calcified, thin peripheral band, often with calcified extensions into the necrotic septa of Bertin.<br />Hyperattenuating tram lines (more commonly interrupted s/o patchiness of CAN)<br />Punctate calcification (CGN)<br />Vishal Golay's<br />
  30. 30.
  31. 31. X-Ray KUB<br />MedullaryNephrocalcinosis<br />Appears as clusters of stippled calcifications, mainly within the regions of the renal pyramids.<br />Many of the disease causing medullary NC also causes nephrolithiasis which can be seen<br />Vishal Golay's<br />
  32. 32. Vishal Golay's<br />Pyramidal calcification in right upper pole<br />
  33. 33. Spiral CT Scan<br />Most sensitive modality.<br />Can pick up diseases at an earlier stage, and better information about the extent of disease.<br />Also picks up other findings eg. Cysts<br />Use of contrast to d/f between stones and true NC, and also for MSK<br />Radiation is an issue (10 times more than KUB X-ray)<br />Vishal Golay's<br />
  34. 34. Vishal Golay's<br />
  35. 35. USG<br />Can pick up NC even at an earlier stage than by X-ray.<br />In medullay ND the pryamids are visualized as rounded or echogenic structures. Shadowing can also be seen.<br />In cortical NC, increased cortical echogenicity and shadowing in severe cases. Secondary pyramidal fibrosis with increased echotexture can be seen.<br />Vishal Golay's<br />
  36. 36. Treatment <br />Hypercalcemic nephropathy: Supportive treatment of the hypercalcemia and treatment of the underlying etiology<br />For the other causes treatment of the underlying cause forms the only possible care<br />Vishal Golay's<br />
  37. 37. Treatment (some examples)<br />Thiazide diuretics and dietary salt restriction<br />Potassium and Mg supplementation<br />Citrate supplementation-increases urinary citrate and decreases Ca excretion (useful in dRTA and idiopathic hypercalciuria)<br />Magnesium supplementation in Mg losing disorders<br />Vishal Golay's<br />
  38. 38. Treatment (some examples)<br />Pyridoxine in Type1 hyperoxaluria.<br />Oral calcium supplementation, low fat diet and cholestyramine in hyperoxaluria to decrease intestinal absorption<br />Alkali supplementation in dRTA<br />Surgical attempts at removing the nodules of NC can cause further destruction and should be avoided<br />Vishal Golay's<br />
  39. 39. THANK YOU<br />Vishal Golay's<br />

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