1. Renal failure typical case reports morphology Doc. MUDr. Zdeňka Vernerová, CSc., MUDr. Martin Havrda

2. Case 1

3. Male, 50 years, complaints: Suddenly –malaise, –fatigue –weakness, –feeling like if he was drunk, –nausea,vomiting, –dizziness, –shortness of breath, Later pain in the loins. Truck driver problems started soon after breakfast. He had breakfast in the truck coffee was somewhat strange and sweet, they cooked it from their own water, stored in a plastic bottle.

4. Male, 50 years, physical examination: Hyperpnoe. Confusion.

5. Male, 50 years, laboratory tests: pH 7,0 HCO3 6,0 mmol/l urea 35 mmol/l creatinine 850 umol/l

6. Male, 50 years, DIAGNOSIS? INTOXICATION WITH ETHYLENE-GLYCOL.

9. Male, 50 years, confirmation of the diagnosis: Severe metabolic acidosis. –With anion gap (Na + K) – (Cl + HCO3) –Glycolic acid Renal failure High serum osmolality –Osmotic gap 2 x (Na+K) + urea + glucose to measured osmolality (N 8-12 mosmol/kg) Hypocalcemia –calcium oxalate Toxicologic tests

10. Male, 50 years, follow-up: Pharmacologic treatment –Ethanol (slowing down the dehydrogenation of ethylene-glycol to glycolic acid) –HCO3 – –Thiamine, pyridoxine Hemodialysis may be necessary –Renal failure –Metabolic acidosis –Elimination of ethylene-glycol Recovery is common.

11. Case 2

12. Male, 21 years, complaints: For 1 month – mild cough with bloody expectoration. Shortness of breath. General malaise, arthralgias. 1 week – dark urine (Coca-Cola colour). Smoker, works in a sawmill – lot of dust.

13. Male, 21 years, physical examination: Pale appearance. Exertional dyspnea. Symmetrical respiratory rales above the lung base.

14. Male, 21 years, laboratory tests: Hgb 60 g/l Urea 50 mmol/l creatinine 1200 umol/l Urinalysis - RBC´s 150/ul Chest X-ray – symmetrical large inexpressive infiltrates of both lungs (reminding butterfly wings)

15. Male, 21 years, DIAGNOSIS? GOODPASTURE´S SYNDROME.

22. Male, 21 years, confirmation of the diagnosis: Anti GBM antibodies in serum Hematuria, proteinuria Renal failure Respiratory failure Anemia Renal biopsy –RPGN with crescents –Linear IF

23. Male, 21 years, follow-up: Plasma exchange daily 20-30x Immun-supressive treatment –Pulse steroids –Cyclophosphamide Supportive treatment Risk of death due to pulmonary bleeding and respiratory failure If remission is achieved, the prognosis is good

24. Case 3

25. Male, 60 years, complaints: In the last 3 months, recurrent treatment for various inflammatory disorders in the ENT area –Otitis media left side –Otitis media right side –Sinusitis –Bloody rhinitis 1 month of coughing, expectoration with the traces of blood, shortness of breath. Fever up to 38,5 deg. Celsius, pain of the muscles and joints, malaise, dyspepsia, sweating. Small red dots on the calf. Small skin ulcers on the skin of the head.

26. Male, 60 years, physical examination: Pale, sweating, exertional dyspnea Purulent ulcers on the head Palpable purpura on the calfs Purulent stomatitis, perforation of the soft plate Purulent secretion from the ear Breathing sounds with wheezing, assymetrical rales Tachycardia Fever 38,2 deg. Celsius

27. Male, 60 years, laboratory tests: ESR 120/150 mm CRP 180 mg/l WBC´s 12,0 (70% neutrophils) Hgb 80 g/l Urea 22 mmol/l creatinine 270 umol/l Urinalysis - RBC´s 25/ul Proteinuria 1,8 g/24h Chest X-ray: Bilateral multiple infiltrates of various size, one with a central cavity.

28. Male, 60 years, DIAGNOSIS? POLYANGIITIS WITH GRANULOMATOSIS (WEGENER´S GRANULOMATOSIS)

34. Male, 60 years, confirmation of the diagnosis: Glomerular hematuria, red blood cell casts Proteinuria Loss of GFR ENT inflammatory involvement, pulmonary C-ANCA positive, Anti PR3 positive Renal biopsy –RPGN with crescents –Pauciimmune (IF negat) Morphologic evidence of vasculitis in some cases

35. Male, 60 years, follow-up: Treatment –Cyclophosphamide –Steroids –Supportive and symptomatic treatment –Plasma exchange in severe cases (cca 10x) Achieving of a remission... Maintanance treatment High risk of relapse Risk of death (renal failure, respiratory failure, complications of treatment, local complications)

36. Case 4

37. Female, 65 years, complaints: 2 month migrating arthralgias Fever up to 38,5 deg. Celsius Weight loss Fleeting skin rash Detection of proteinuria and hematuria Weakness and tingeling in both legs Progressive paralysis of both legs, inability to walk, weakness of both arms

38. Female, 65 years, physical examination : Quadruparesis Palpable purpura on the calfs Pale, sweating

39. Female, 65 years, laboratory tests : ESR 120/150 mm CRP 150 mg/l Urea 20 mmol/l creatinine 230 umol/l Urinalysis - RBC´s 35/ul Proteinuria 1,2 g/24h WBC´s 12,3 75% neutrophils Hgb 80 g/l

40. Female, 65 years, DIAGNOSIS? MICROSCOPIC POLYANGIITIS.

45. Female, 65 years, confirmation of the diagnosis: Glomerular hematuria. Proteinuria. Loss of GFR. P-ANCA positive, Anti MPO positive. Peripheral polyneuropathy. Renal biopsy –RPGN with crescents –Pauciimmune (IF negative)

46. Female, 65 years, follow-up: Treatment –Cyclophosphamide –Steroids –Supportive and symptomatic treatment –Plasma exchange in severe cases (cca 10x) Achieving of remission... maintanance treatment Risk of relapse Risk of death (renal failure, organ complications, complications of treatment)

47. Case 5

48. Male, 50 years, complaints: Headache Visual impairment Confusion Dyspnea

49. Male, 50 years, physical examination: BP 260/150 mmHg Signs of cardiac failure –Rales above the lung base –Tachycardia

50. Male, 50 years, laboratory check: Urinalysis - RBC´s 40/ul Proteinuria 2,5 g/24h Urea 25 mmol/l, creatinine 450 umol/l

51. Male, 50 years, DIAGNOSIS? MALIGNANT NEPHROSCLEROSIS. (malignant hypertension)

56. Male, 50 years, confirmation of the diagnosis? Eye fundus – hypertensive changes (retinal bleeding, papiledema). Hematuria, proteinuria, loss of GFR. EKG, ECHO signs of LV hypertrophy. Renal biopsy. –Fibrinoid necrosis of preglomerular arterioles.

57. Male, 50 years, follow-up: Treatment –Lowering the BP –Dialysis in some cases Prognosis –Improvement in renal function may occur –Irreversible renal failure in come cases –Commonly cardiovascular complications

58. Case 7

59. Male, 60 years, complaints: Proteinuria and elevated s-creatinine found during preventive check. Mild fatigue, no other serious symptoms. 10 years ago already proteinuria was discovered, not checked precisely. 10 years ago the diagnosis of hypertension.

60. Male, 60 years, physical examination: Pale. BP 160/100 mmHg.

61. Male, 60 years, laboratory check: Urea 30 mmol/l, creatinine 630 umol/l Hgb 85 g/l S-Ca 1,8 mmol/l, s-P 2,4 mmol/l S-PTH 65 pmol/l pH 7,23 HCO – 11 mmol/l

62. Male, 60 years, DIAGNOSIS? End stage kidney Etiology unknown –Vascular nephrosclerosis? –Chronic glomerulonephritis? –Another reason?

65. Male, 60 years, confirmation of the diagnosis? Shrinking of the kidneys, –Bumpy contours, –Thin parenchymal layer, –Loss of architecture (sonography). Abnormalities characteristic for chronic renal failure –Anemia –Hypocalcemia, hyperphosphatemia, hyperparathyreoidism –Renal osteodystrophy (Rtg) Renal biopsy in case of doubt.

66. Male, 60 years, follow-up: Stabil clinical condition → conservative treatment –Correction of hypertension –Compensation of metabolic abnormalities Anemia Acidosis Ca-P-PTH metabolism –ACEi –Prepare the patient for renal replacement therapy Unstable clinical condition → renal replacement therapy Prognosis severe – cardiovascular complications