November Board Review

1. Renal: Part I November Board Review

2. Test Question • You like having 15 questions during board review instead of 10. A. True B. False

3. Hematuria

4. Question #1 • The mother of a 5-year-old female brings her daughter to your clinic on Monday morning because when she went potty this morning her urine was red. She is afebrile and has no complaints of dysuria or musculoskeletal pain. Her BP is 94/55 and her urinalysis is negative for blood, protein, nitrites, and leukocyte esterase. Microscopy reveals 1 RBC and no casts. You notice scratches on the girls fingers, and her mother reports that they have just returned from a friends farm and went “berry pickin” over the weekend. • Of the following, the next MOST appropriate step is: • A. Reassurance • B. Renal U/S • C. Creatininekinase • D. BMP • E. Urine culture

5. Hematuria • 5 or more RBCs per high-power field in 3 consecutive fresh, centrifuged specimens obtained over the span of several weeks • (+) dipstick for heme • Myoglobinuria • Hemoglobinuria • (-) dipstick for heme • Drugs (sulfa, nitrofurantoin, salicylates, phenazopyridine, phenolphthalein) • Toxins (lead, benzene) • Food (food coloring, beets, blackberries, rhubarb, paprika)

6. Glomerular Bleeding • Discolored urine • RBC casts • Distorted RBC morphology

8. Causes

9. Gross Hematuria • Underlying cause is found in 56% of cases • Common causes: • UTI • Trauma • Coagulopathy • Crystalluria • Nephrolithiasis

10. Question #2 • The mother of a 3-year-old male comes to clinic for follow-up of hematuria. The results of today’s UA are: • pH 6.0 • 3 + blood • Negative for protein, nitrite, and leukocyte esterase • 10 to 20 RBCs/HPF • 0 WBCs These results are similar to the UA you performed 2 weeks ago. The patients BP is 105/58, but otherwise the physical exam is normal. Being the astute clinician that you are, you ask mom about any family history of renal disease. She mentions that both of her older brothers are on dialysis. • Of the following, the MOST likely cause of this patient’s hematuria is: • A. Benign familial hematuria • B. Polycystic kidney disease • C. Alport syndrome • D. Sickle cell disease • E. Ureteropelvic junction obstruction

12. Family History is Important! • Alport syndrome • X-linked (85%), autosomal recessive, and autosomal dominant • Mutation in Type IV collagen of GBM • Kidney failure by 2nd or 3rd decade in males • Sensorineural hearing loss • Anterior lenticonus • Females may only have microhematuria

13. Family History is Important! • Benign familial hematuria • Autosomal dominant • But many people are unaware • Also mutation in type IV collagen of GBM • Microscopic hematuria with occasional gross episodic hematuria (<10%) • Proteinuria and HTN are unusual

14. Family History is Important! • Sickle cell disease and trait • Occlusion of the vasa recta capillaries result in renal infarct • Hematuria more common in males • Unilateral, left more common • Recurrence in 40% of cases • Contributing factors • Hypoxia • Acidosis • High osmolality • Stasis

15. Symptomatic Microscopic Hematuria • If accompanied by elevated proteinuria on first morning urine → higher likelihood of underlying renal disease • Clinical manifestations • Nonspecific • Fever, malaise, weight change • Extrarenal • Malar rash, purpura, arthralgia/arthritis, headaches • Localized • Dysuria, suprapubic pain, flank pain, edema, oliguria

16. Question #3 • The results of a urinalysis for a 13-year-old male in your practice reveal: • pH 6.0 • 3+ blood • Negative glucose, protein, nitrite, and leukocyte esterase • 5 to 10 RBCs/HPF • He has never noticed any changes in his urine, has no complaints, takes no medications, and has no family history of kidney disease. • Of the following, the MOST likely associated urinary finding is: • A. Bacteriuria • B. Hypercalciuria • C. Hemoglobinuria • D. Protein-to-creatinine ratio 1.0 • E. Myoglobinuria

17. Isolated Asymptomatic Hematuria • Rarely have significant renal disease • 25% normalize within 5 years • Rarely have gross hematuria • Get good family history! • Monitor for HTN and proteinuria • Hypercalciuria • Risk for urolithiasis • Urinary Ca/Cr > 0.2 or 24 hour urinary calcium excretion > 4 mg/kg/day • Mostly idiopathic • Consider immobilization, diuretics, vit D intoxication, hyperparathyroidism, and sarcoidosis

18. Question #4 • A 14-year-old female has 3+ blood, 2+ protein, and 10 to 20 RBCs/HPF on urinalysis. She has no symptoms. • Of the following, the BEST next step is: • A. Refer to nephrology • B. Renal U/S • C. Urine culture • D. Urinary calcium-to-creatinine ratio • E. Repeat UA with first morning urine

19. Asymptomatic hematuria + proteinuria • In most cases, resolution of one or both features • Determine if proteinuria is orthostatic • First morning urine • Persistent proteinuria is more indicative of a glomerular process • Refer to nephrology

20. Diagnostic Evaluation

21. Diagnostic Evaluation • First stage • BP, UA +/- urine culture • Second stage • Search for underlying disease especially if edema, HTN, systemic symptoms, etc. • BMP • ASO • Complement • ANA • Hepatitis panel • HIV • CBC • Hgb electrophoresis • Renal U/S, biopsy, cystoscopy when indicated • Refer to nephrology unless the cause is clear (UTI, postinfectious)

22. Question #5 • An 8 yo M presents to the ED with c/o “dark urine” for the past 24 hours. Per Mom, this has never happened before. He is a very healthy boy, with the exception of being treated with antibiotics for a sore throat 2 weeks ago. On exam, his BP was found to be 130/82, and there was some mild periorbital edema bilaterally. Of the following, the most likely set of laboratory findings in this patient is: • A. Normal complement levels; 3+ protein, no blood on UA • B. Elevated complement levels; 1+ protein, 1+ blood on UA • C. Low complement levels; trace protein, 3+ blood on UA • D. Low complement levels; 3+ protein, no blood on UA • E. Normal complement levels; no blood or protein on UA

23. Acute poststreptococcalglomerulonephritis (PSGN)

24. Pathophysiology • Caused by a reaction to a nephritogenic strain of group A beta-hemolytic Streptococcus • Multiple pathogenic mechanisms inflammation of the glomeruli • Deposition of Ag-Ab complexes in the glomeruli • In situ deposition of nephritogenic Ag with formation of immune complexes • Direct activation of complement by the nephritogenic Ag within the glomeruli

25. Presentation • Timeline • 10-14 days after pharyngitis • 3-6 weeks after pyoderma • Clinical features • Classic nephritic syndrome • Gross hematuria • Edema (fluid overload can pulmonary edema) • HTN • Renal insufficiency

26. Laboratory Findings • Low C3 • UA • Hematuria* • Proteinuria • RBC casts • BMP • Mild renal insufficiency • Evidence of past streptococcal infection • Streptozyme • Anti-DNAase B

27. Question #6 • Of the following, which most accurately describes the natural course of PSGN: • A. Renal function improves within 3 weeks, complement values normalize within 8-12 weeks • B. Renal function improves within 6-24 months, complement values normalize within 8-12 weeks • C. Renal function improves within 3 weeks, complement values remain low indefinitely • D. Renal function continues to deteriorate, complement levels remain low indefinitely • E. Renal function improves within 3 weeks, complement values normalize within 2-4 weeks

28. Treatment and Prognosis • Supportive management • Antibiotics (Rx Strep infection) • For Fluid overload: • Fluid/ Na restriction • Diuretic therapy • Monitor electrolytes and renal function • Anti-hypertensives

29. Treatment and Prognosis • Prognosis is excellent! • Renal function improves within 3 weeks • Complement values normalize within 8-12 weeks • Microscopic hematuria may persist 6-24 months • Indications for renal biopsy: • Renal function or BP abnormal for >4wks • Proteinuria present for >6mos • Serum complement concentrations remain low for >12wks

30. Enuresis

31. Definitions • Primary nocturnal enuresis = nighttime wetting in a child who has never been dry on consecutive nights for longer than 6 months • Dryness is expected to be achieved by 5 years of age • 10 to 15% of 7-year-olds still have bedwetting • 99% of children are dry by 15 years • Incontinence = uncontrollable leakage of urine that may be intermittent or continuous and occurs after continence should have been achieved • Leakage that occurs during the day is daytime incontinence • Dysfunctional voiding = inappropriate muscle contraction during voiding

32. Question #7 • A 7-year-old boy has nighttime bedwetting. No one else in the family wet the bed, but his mother his concerned about his weight and that he is constantly tired during the day. Other than being overweight, his physical exam and screening UA are normal. • The MOST likely cause of his nocturnal enuresis is: • A. Genetic predisposition • B. Bladder dysfunction • C. He is a “deep” sleeper • D. Occult spinal dysraphism • E. Obstructive sleep apnea

33. Causes of Nocturnal Enuresis • No data to support “deep” sleep theory • Obstructive sleep apnea • ↑ atrialnatriuretic factor leads to increased diuresis • T & A has been shown to cure enuresis • Abnormal circadian release of ADH

34. Causes of Nocturnal Enuresis • Bladder dysfunction • Smaller-than-normal functional bladder capacity at night • Higher bladder instability • Daytime + Nighttime = higher degree of abnormalities and treatment failure

35. Causes of Nocturnal Enuresis • Genetics • 1 parent with enuresis = 44% chance of child affected • 2 parents with enuresis = 77% chance of child affected • Psychological factors • 30% greater chance of enuresis in kids with ADHD • Maturational delay • Fine and gross motor clumsiness, perceptual dysfunction, speech defects co-exist

36. Secondary Enuresis • New-onset nighttime wetting on consecutive nights after a 6-month or greater period of dryness • Usually not related to an organic cause • Stressful events can be the source • Birth of a sibling, move, death in the family

37. Evaluation

38. Question #8 • An 8-year-old female is diagnosed with primary nocturnal enuresis. The parents are interested in therapy. • The MOST effective treatment for ending the enuresis is: • A. Desmopressin • B. Alarm therapy • C. Imipramine • D. Anticholinergics • E. Limiting dairy products before bed

39. Treatment of Nocturnal Enuresis

40. Alarm Therapy • Most effective • Success rates as high as 66 to 70% • Most difficult to employ • Must be used every night • Requires 3 to 4 months for results • Parents may need to wake up child if the child does not wake to alarm • Offers a real cure • No adverse effects

41. Daytime Wetting • Can be caused by stressful events • Divorce, death in the family, abuse • Children with daytime wetting may have a difficult temperament • Increased risk for constipation and encopresis

43. Classification of Daytime Wetting • Storage problem • Neurologically normal who cannot fill and store • Neurologically abnormal who have high pressure bladder • Hypersensitive bladder • Inadequate sphincter tone • Emptying problem • Failure to empty completely with little residual urine • May be neurologic, anatomic, muscular, or fucntional

44. Question #9 • A 7-year-old female patient has nighttime and daytime wetting. You order a UA and urine culture. Her physical exam is normal. • Which of the following should also be included in your initial evaluation? • A. Post-void residual • B. VCUG • C. Urodynamics • D. MRI of the spine • E. Cystoscopy

45. Evaluation

46. Ectopic Ureter • Females • No history of day- or nighttime dryness • “Constant dribbling” • Evaluate with MR urography, CT, or IVP • Refer to pediatric urology

47. Manifestations of Storage Problems • Urge incontinence • Frequent attacks of a strong desire to urinate countered by hold maneuvers such as squatting, dancing, and curtseying • Uninhibited bladder contractions • Dampness rather than soaking • Functional bladder capacity is usually small

48. Storage Problems • Overflow incontinence • Infrequent and incomplete voiding • Overtime decreased sensation of the need to void • Usually large wetness • Daytime incontinence • Infrequent or delayed voiding, especially associated with distraction or play • Small to large urine loss • Associated with behavior problems • Development after continence should prompt referral

49. Storage Problems • Urinary frequency • Sudden need to urinate very frequently, sometime up to 30 times per day • Ages 3 to 8 • Self-limited • Related to psychological stressors

50. Manifestations of Emptying Problems • Lazy bladder syndrome • Void 3 or fewer times a day • Must strain abdominal muscles to void • Intermittent stream and cannot empty bladder completely • Recurrent UTIs • Constipation • Detrusor sphincter dyssynergia (DSD) • Inappropriate contraction of the external urethral sphincter during bladder contraction • Staccato type of voiding • Post-void residual • “spinning-top urethra”