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Title: Metro New York / New Jersey Pediatric Board Review Course Pediatric Nephrology June 2013


1
Metro New York / New Jersey Pediatric Board
Review CoursePediatric NephrologyJune 2013
  • Leonard G. Feld MD PhD
  • Levine Childrens Hospital
  • Charlotte, NC

2
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3
For the Exam
  • Fluid and Electrolyte Metabolism
  • A. Composition of body fluids -Intracellular,
    extracellular, Electrolytes (sodium, potassium,
    chloride), Protein
  • B. Acid-base physiology -Normal mechanisms and
    regulation, Acidosis, alkalosis
  • C. Electrolyte abnormalities - Sodium
    Hypernatremia Hyponatremia Potassium
    Hyperkalemia Hypokalemia Chloride imbalance
  • D. Disease states, specific therapy - Pyloric
    stenosis, gastroenteritis, acute renal failure,
    shock, SIADH, Cystic fibrosis, Dehydration,
    Hyperosmolar non-ketotic coma

4
For the Exam
  • Renal Disorders
  • Normal function, proteinuria, hematuria,
    persistent microscopic hematuria, causes of gross
    and microscopic hematuria, nonhematogenous
    etiology of red urine,dysuria,
  • Incontinence - Nocturnal, functional, daytime
    incontinence,
  • Congenital - Renal dysplasia, unilateral
    multicystic dysplastic kidney, autosomal dominant
    polycystic kidney disease, autosomal recessive
    polycystic kidney disease, Juvenile
    nephronophthisis, Renal agenesis
  • Abnormalities of the collecting system, kidney,
    and bladder Hydronephrosis, Hydroureter and
    megaureter, Ureterocele, Vesicoureteral reflux,
  • Abnormalities of the urethra - Posterior
    urethral valves, Urethral stricture
  • Hereditary nephropathy - Familial nephritis,
    Congenital nephrotic syndrome
  • Acquired - Infection of the urinary tract
    (Pyelonephritis, Cystitis), acute
    glomerulonephritis, Nephrotic syndrome,
    Hemolytic-uremic syndrome, Henoch-Schoenlein
    purpura,IgA nephropathy
  • Renal failure - Prerenal failure, Intrinsic renal
    failure , chronic renal failure,
  • Other - Trauma, renal injuries, Urethral injury,
    Toxins, Renal stones, Renal tubular acidosis,
    Hereditary conditions with renal manifestations,
    Nephrogenic diabetes, insipidus, Cystinosis
  • Hypertension General, Renal, Vascular, Adrenal,
    Pheochromocytoma, Cushing syndrome, Miscellaneous
    causes, Essential hypertension, Administration of
    drugs, Traction on legs

5
ANSWERS TO THE CONTENT SPECIFICATIONS
  • Fluid and electrolyte
  • Nephrology
  • Leonard.feld_at_carolinashealthcare.org
  • SUBJECT LINE NYNJ PEDS

6
Outline
  • Fluid and Electrolyte abnormalities
  • Dehydration
  • Renal
  • Hematuria
  • Proteinuria
  • Hypertension
  • Urinary tract infections
  • Glomerulonephritis

7
Case 1 Dehydration
A 4 mo old infant presents with a four to five
day history of low grade fever (38-38.5oC),
numerous watery diarrhea and decreased activity.
Since the child refused to take her usual breast
milk volume or solid foods, the mother and
grandmother substituted non-carbonated soda
(Coca-cola, ginger ale, apple juice or orange
juice will have 550-700 mOsm/kg H2O) with less
than 5 mEq/L of sodium), and sweet (sugar
added) iced tea. Over the last 12 hours there
were a few episodes of emesis and there were less
wet diapers. On examination the child was
lethargic, dry mucous membranes, no tears, sunken
eyeballs, and reduced skin turgor. Vitals signs
were the following Blood pressure 74/43 mmHg
Temperature of 38oC, respiratory rate of 36 per
minute and pulse of 175 beats per minute. The
weight was 6 kg. Weight at the time of her
immunization 7 days ago was 6.6 kg. There were no
other significant findings.
8
Electrolyte Composition of Body Fluid Compartments
9
BODY WATER DISTRIBUTION
10
Clinical Assessment
Fluid Deficit Clinical Status Clinical Assessment
Mild ( 5) 50 cc/kg Compensated Thirsty,? HR, Normal BP tears, slightly dry mucosa, alert/restless, urine
Moderate (10) 100 cc/kg Decompensated Very dry mucosa, lt skin turgor, sunken eyes, deep resp, weak pulses, cool extremities, oliguria
Severe (15) 150 cc/kg Shock Intense thirst, ? BP, cap refill gt 3 sec, weak pulses, apnea/rapid breathing, coma, anuria
11
Maintenance Requirements
Body wt 0-10 kg 10-20 kg 20 kg
TBW 100 ml/kg 1000 ml 50 ml/kg for each kg gt 10kg 1500 20 ml/kg for each kg gt 20kg
Na 3 mEq/kg 3 mEq/kg 3 mEq/kg
K 2 mEq/kg 2 mEq/kg 2 mEq/kg
Cl- 5 mEq/kg 5 mEq/kg 5 mEq/kg
12
Deficit
Type of Dehydration based on serum Na in mEq/L Water (mL/kg) Sodium (mEq/kg) Potassium (mEq/kg)
Isonatremic Na 130-150 100-120 8-10 8-10
Hyponatremic Na lt 130 100-120 10-12 8-10
Hypernatremic Na gt 150 100-120 2-4 0-4
13
Laboratory
Sodium 124 mEq/L, chloride 94 mEq/L normal 98
-118 mEq/L), potassium 4 mEq/L (normal 4.1-5.3
mEq/L), bicarbonate (or total CO2) was 12 mEq/L
(normal 20-28 mEq/L or mmol/L), serum creatinine
0.8 mg/dL (normal 0.3-0.5 mg/dL), blood urea
nitrogen 40 mg/dL, blood glucose 70 mg/dL
complete blood count was normal except for a
hemocrit of 38 (normal 36)
14
Hyponatremia
  • Serum Na lt 130 mEq/L
  • Water shifts into cells lower ECF volume
  • lt125 mEq/L nausea and malaise
  • lt 120 mEq/L headache, lethargy,
  • lt115 mEq/L seizure and coma

15
Loss of hypertonic Fluid and Sodium from the ECF
secondary to Dehydration
16
Question 1 What is the appropriate parenteral
solution
  1. 5 dextrose 0.45 isotonic saline 40 mEq KCl
    /L
  2. 0.45 isotonic saline 40 mEq KCl /L
  3. 0.9 isotonic saline 40 mEq KCl /L
  4. 5 dextrose 40 mEq KCl /L
  5. 5 dextrose 0.2 isotonic saline

17
Approach
For this 6.6 kg infant Maintenance requirements
for 24 hours Water 100 mL / kg x 6.6 kg 660
mL Sodium 3 mEq / 100 mL x 660 mL 20
mEq Potassium 2 mEq / 100 mL x 660 mL 13 mEq
For this 6 kg infant with hyponatremic
dehydration at 10 Deficits for 24 hours Water
Pre-illness weight Illness weight 6.6
6 kg 0.6 kg 600 mL Sodium 10 mEq x 6.6 kg
66 mEq Potassium 8 mEq x 6.6 kg 53 mEq
18
Points on Hypernatremic Dehydration
19
Hypernatremia SLOW and CLOSE
  • Key points look quiet then irritable on
    stimulation may look better than of
    dehydration based on weightd
  • Fluid selection 5 dextrose ¼ isotonic saline
    (30-40 mEq/L of Na) 20 mEq KCl /L
  • 1st 24 hrs 24 hrs of Maintenance ½ deficit
  • 2nd 24 hrs 24 hrs of Maintenance ½ deficit
  • Close monitoring of serum sodium every 2-3 hours.
  • Some have suggested using a higher sodium
    0.45 isotonic saline or even isotonic saline to
    restore ECF volume then moving to a lower
  • sodium containing solution to restore the water
    deficit. This approach may also reduce the
    possibility of dropping the serum sodium too
    quickly and preventing neurological problems.

20
Hematuria
  • Case Susan is an 8 year old noted on routine
    exam to have moderate hematuria on dipstick. She
    has an unremarkable past medical history. Family
    history is negative in the parents and siblings
    for any renal disease. History of hematuria is
    unknown. A repeat urine in one week is still
    positive and a urine culture showed no growth.

21
Question 2 Which of the following test is the
next step in the evaluation?
  1. VCUG and urine culture
  2. Renal sonogram and urine calcium to creatinine
    ratio
  3. Urology referral
  4. CBC and Direct Coombs
  5. Recheck in two years

22
More on Hematuria
  • Repeat a first AM void following restricted
    activity , perform a microscopic on a fresh urine
  • Check the family members
  • If there is still blood without protein, casts,
    crystals, normal BP with or without a strong
    family history, no further work-up is generally
    required. However a renal sonogram and urine
    calcium to creatinine ratio
  • Caveat - Family anxiety because of the
    connotation of blood and cancer in adults.

23
Glomerular v. Non-glomerular bleeding
  • Glomerular
  • oliguria, edema, hypertension, proteinuria,
    anemia
  • Non-glomerular
  • dysuria, frequency, polyuria, pain or colic, hx
    exercise
  • crystals on microscopic
  • mass on exam
  • medication history - sulfas, aspirin, diuretics

24
Initial evaluation of the patient with hematuria
  • All patients BUN, creatinine, kidney and bladder
    ultrasound, urine calcium to creatinine ratio
  • Who should be worked up
  • Presence of proteinuria and/or hypertension ,
  • History consistent with infectious history, HSP,
    systemic symptoms, medication use or abuse,
    strong family history of stones or renal
    disease/failure.
  • Persistent gross hematuria
  • Family anxiety - limit evaluation
  • Probable glomerular hematuria
  • C3, ASO titer
  • possible hepatitis, HIV, SLE serology , SSD
  • renal biopsy not for persistent microscopic
    without proteinuria, decreased renal function,
    and/or hypertension
  • Probable non-glomeurlar hematuria
  • urine culture, urine Ca/creatinine ratio
  • possible hemoglobin electrophoresis,
  • coagulation studies, isotope scans,
  • Flat plate, CT, ??IVP, cystoscopy

25
Pearls for Hematuria
  • Hematuria may be an important sign of renal or
    bladder disease
  • Proteinuria (as we will discuss) is the more
    important diagnostic and prognostic finding.
  • Hematuria almost never is a cause of anemia
  • The vast majority of children with isolated
    microscopic hematuria do not have a treatable or
    serious cause for the hematuria, and do not
    require an extensive evaluation. So a VCUG, cysto
    and biopsy are not indicated.

26
More Pearls
  • Urethrorrhagia boys with bloody spots in the
    underwear
  • Presentation prepuberal 10 yrs
  • It is painless
  • Almost 50 will resolve in 6 months and gt 90 at
    1 year it may persist for 2 yrs
  • Treatment watchful waiting in most cases
  • Painful gross hematuria usually infection,
    calculi, or urological problems glomerular
    causes of hematuria are painless.

27
More Pearls gross hematuria
  • Gross hematuria is often a presentation of Wilms
    tumor
  • All patients with gross hematuria require an
    imaging study.
  • If a cause of gross hematuria is not evident by
    history, PE or preliminary studies, the
    differential includes hypercalciuria or SS trait
  • Cysto is rarely helpful

28
Trying to think about pediatric kidney disease -
nephWHAT
29
Case 3
  • 7 year old boy developed gross tea colored
    hematuria after a sore throat and upper
    respiratory infection. No urinary symptoms but
    urine output was decreased. He complained of
    mild diffuse lower abdominal pain. There is no
    fever, rash or joint complaints. Past med history
    was unremarkable but had intermittent headaches
    for two years.
  • On exam he was well (afebrile) with a BP of
    95/65 mHg, no edema, some suprapubic tenderness
    and red tympanic membranes. The mother thinks
    that a similar episode occur on vacation a few
    months ago.
  • Urinalysis shows 20 RBCs/hpf, 5-10 WBCs, 100
    mg/dL of protein, rare cellular and hyaline
    casts. Serum creatinine is 0.8 mg/dL, C3 100
    (normal).

30
Question 3 The most likely cause of the gross
hematuria is
  1. Myoglobinuria
  2. Urinary tract infection
  3. Obstructive uropathy
  4. IgA nephropathy
  5. Benign familial hematuria

31
IgA
  • IGA nephropathy
  • Boys gt girls
  • Mostly normotensive, with persistent microscopic
    hematuria
  • Chronic glomerulonephrits up to 40 of primary
    glomerulonephritis
  • Complement studies are nl, some inc IgA
  • Prognosis not so good if gt 10 yrs of age,
    proteinuria, reduced GFR, hypertension and no
    macrohematuria

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Glomerular Non-glomerular
Urinalysis Dysmorphic RBC Cellular casts Brown/tea color Bright red Clots Crystals Protein - - - - -
History Family Hx of ESRD Systemic disease Nephrolithiasis Trauma Symptomatic vomiting - - - - -
Physical Hypertension Systemic signs Edema Abdominal mass Genital bruising - - - -
34
Red or Tea colored/ Brown Urine
Fresh Centrifuged Urine Sample
Sediment Red with Red Cells
Supernatant Red without Red Cells
Hemoglobinuria Myoglobinuria
Hematuria
Hemoglobinuria will have a red or pink hue to
the serum
NOTE If there is no red sediment, no RBCs and a
clear supernatant, consider other causes such as
urates, bile pigments, beets, porphyria, some
medications, etc.
35
Question 4
  • On routine physical examination, an 8-year-old
    boy is found to have microscopic hematuria. The
    first step in your evaluation should be.
  • Examine the urine sediment
  • Order an renal ultrasound
  • Obtain a voiding cystourethrogram
  • Perform a CBC in the office
  • Order an ASO titer and C3

36
Question 5
  • An 8-year-old boy presents with tea colored
    urine. He has very mild edema. History of strep
    infection about 2 weeks ago. The work-up should
    include all the following except.
  • Complement studies
  • Serum creatinine
  • Urinalysis for protein
  • Monitor blood pressure and urine output
  • Obtain a renal ultrasound

37
Acute glomerulonephritis clinical
  • May be clinically asymptomatic (? 90) with low
    C3 and hematuria
  • Usually within 3 weeks after strep infection
    mean about 10 days
  • Periorbital, peripheral edema
  • Hematuria - coke-colored, tea-colored,
    reddish/brown
  • Nonspecific findings such as abdominal pain,
    malaise, anorexia, headaches, pallor

38
Acute glomerulonephritis DD
  • Acute Poststreptococcal glomerulonephritis
    (PSAGN) most common
  • Acute Postinfectious or nonstreptococcal
    postinfectious glomlerulonephritis 9AIAGN)
  • Bacterial endocarditis (low C3), shunt nephritis
    (low C3), pneumococcal pneumonia, etc.
  • Viral hepatitis B, infectious mononucleosis,
    varicella, etc,
  • Parasites
  • Other SLE (low C3), membranoproliferative GN
    (low C3), hyperthyroidism, HSP (nl C3)

39
Acute glomerulonephritis evaluation/ treatment
  • Evaluation
  • ASO, C3, C4
  • Renal function
  • Evaluation for hypertension and oliguria
  • Magnitude of proteinuria
  • RX supportive
  • Admission for hypertension, oliguria, impaired
    renal function, nephrotic syndrome
  • Prognosis C3 normalizes by 12 weeks,
    hypertension and other abnormalities resolve by
    2-3 months, hematuria may persist for 6-24 mo

40
SO you call this urinary frequency
41
Proteinuria
  • Case 6 John is an 12 year old noted on a
    basketball team physical to have 2 protein on
    dipstick. There are no recent illnesses. He has
    an unremarkable past medical history and he is
    not taking any medications. Family history is
    negative in the parents and siblings for any
    renal disease.

42
Question 6 Which of the following is the best
approach?
  1. Obtain a 1st AM urine for protein
  2. Perform a complete biochemical profile
  3. Obtain a C3, ASO and ANA
  4. Refer for a renal biopsy
  5. Schedule a renal sonogram and VCUG

43
What does Orthostatic Proteinuria mean?
Protein Excretion
44
More on Proteinuria
  • Repeat a first AM void following restricted
    activity, perform a microscopic on a fresh urine
    also an alkaline pH may give a false positive
    result
  • If there is still protein perform a more formal
    orthostatic test. If orthostatic, no further
    work-up is generally required, although no
    indemnification from subsequent renal disease.

45
Causes of Proteinuria
  • Transient
  • fever, emotional stress, exercise, extreme cold,
    abdominal surgery, CHF, infusion of epinephrine
  • Orthostatic
  • Transient or fixed / reproducible
  • Persistent
  • Glomerular disease MCNS, FSGS, MPGN, MN
  • Systemic SLE, HSP, SBE, Shunt infections
  • Interstitial reflux nephropathy, AIN,
    hypoplasia,
    hydronephrosis, PKD

46
Question 7
  • A four-year boy presents with a 5-day history of
    swollen eyes and larger ankles. On exam he has
    periorbital and pretibial edema. The most
    appropriate tests include all the following
    except.
  • Urinalysis
  • Blood tests for total protein and albumin
  • Serum creatinine
  • Sedimentation rate
  • Serum complement (C3)

47
Definitions (Pearl)
  • Urine protein to creatinine ratio
  • Normal lt 0.2 (lt 0.15 adolescents)
  • Mild to moderate 0.2 to 1.0
  • Heavy or severe gt 1.0
  • Persistent proteinuria present both in the
    recumbent and the upright posture even in this
    situation, proteinuira is less during recumbency

48
Nephrotic Syndrome
  • Primary Nephrotic Syndrome
  • Minimal change disease (75) mean age 4 yrs
  • No hematuria, nl C3, no hypertension, nl
    creatinine
  • Membranoproliferative GN ( 5-10)
  • FSGS (5-10)
  • Proliferative GN, Mesangial proliferation
  • Membranous nephropathy
  • Secondary Nephrotic Syndrome
  • SLE, HSP, diabetes mellitus, HIV, vasculitis,
    malignancy (lymphoma, leukemia), drugs (heroin,
    inteferon, lithium), infections (toxo, CMV,
    syphilis, hepatitis B and C)etc.
  • Congenital/Infantile Nephrotic Syndrome
  • Finnish-type congenital nephrotic
    syndrome,Denys-Drash syndrome
  • Diffuse mesangial sclerosis, Nail-patella
    syndrome

49
Nephrotic Syndrome - RX
  • Corticosteroid treatment
  • Induction therapy
  • Exclude active infection or other
    contraindications prior to steroid therapy.
  • Oral prednisone or prednisolone at 60 mg/m2/d (2
    mg/kg/d) daily for 4 weeks.
  • Maintenance therapy (following above induction
    therapy)
  • Oral prednisone or prednisolone at 40 mg/m2 (or
    1.5 mg/kg) given as a single dose on alternate
    days for 4 weeks.
  • NOTE Some nephrologists recommend daily
    induction steroid treatment for 6 weeks, followed
    by alternate day maintenance therapy for another
    6 weeks.
  • Relapse therapy
  • For infrequent relapses, Prednisone 60 mg/m2/d
    (2 mg/kg/d) given as a single morning dose until
    proteinuria has resolved for at least 3days.
  • Following remission of proteinuria, prednisone is
    reduced to 40 mg/m2 (or 1.5 mg/kg) given as a
    single dose on alternate days for 4 weeks.
    Prednisone may then be discontinued or a tapering
    regimen.
  • Frequently relapsing nephrotic syndrome is
    defined as steroid-sensitive nephrotic syndrome
    with 2 or more relapses within 6 months or more
    than 3 relapses within a 12-month period.

50
Hypertension
51
Hypertension
  • Question 8 David is a 10 year old boy first
    noted to have an elevated blood pressure of
    123/85 during a annual physical examination. Pt
    has a long history of learning and behavioral
    issues. He has a previous history of headaches
    that were evaluated with a CT scan of the brain
    and sinuses. On following evaluation in one week,
    his BP is126/86 mmHg with a weight gt 99ile for
    age and a height at 50th ile.

52
Question 8 What is the most appropriate initial
testing for this child?
  1. Renal mag-3 flow scan
  2. Electrolytes, BUN, Creatinine, Bicarbonate
  3. Renal Sonogram with doppler
  4. Urinary screening for drugs
  5. 24 hour urine for catecholamines

53
BP Classification
Grade of hypertension Definition Appropriate next step
White-coat hypertension BP levels gt95th percentile in a physician's office or clinic, but normotensive outside a clinical setting Readings may be obtained at home with appropriate family training or with the assistance of a school nurse, or with the use of ambulatory BP monitoring (ABPM)
Normal lt 90th ile
Pre-hypertension gt120/80 mm Hg should be considered pre-hypertensive or 90-95ile Additional readings may be obtained at home with appropriate family training or with the assistance of a school nurse
Stage I hypertension 95th -99th ile 5 mmHg Organize a diagnostic evaluation in a non-urgent, phased approach
Stage II hypertension Average SBP or DBP that is gt5 mm Hg higher than the 99th percentile Organize a diagnostic evaluation over a short period of time in conjunction with pharmacological treatment
Hypertensive urgency and emergency Average SBP or DBP that is gt5 mm Hg higher than the 95th percentile, along with clinical signs or symptoms Hospitalization and treatment to lower the blood pressure
54
Estimate of Hypertension
  • Estimate without height adjustment
  • If systolic BP equals or exceeds
  • 100 2 times pt age in yrs
  • If diastolic BP equals or exceeds
  • 70 pt age in yrs
  • Estimate with height adjustment
  • If systolic BP at 95th tile for age and sex
  • Add 4 mmHg to the value at the 50th tile
  • 2. If diastolic BP at 95th tile for height
  • Add 2 mmHg to the value at the 50th tile

55
Evaluation of Hypertension
56
Indications for Treatment
  • Symptomatic hypertension
  • Secondary hypertension
  • Hypertensive target-organ damage
  • Diabetes (types 1 and 2)
  • Persistent hypertension despite nonpharmacologic
    measures

57
Pharmacologic Therapy for Childhood Hypertension
  • The goal for antihypertensive treatment in
    children should be reduction of BP to lt95th
    percentile, unless concurrent conditions are
    present. In that case, BP should be lowered to
    lt90th percentile.
  • Severe, symptomatic hypertension should be
    treated with intravenous antihypertensive drugs.

58
Urinary Tract Infections
59
Case 9
  • A 4 mo old girl presents with low grade fever,
    mid-lower abdominal pain and nighttime-incontinenc
    e. She is not eating well. Prior visits she had
    normal blood pressure, urinalysis and excellent
    growth. Urinalysis shows hematuria, 30 mg/dL of
    protein, leukocyte esterase and positive nitrite.
    Urine culture is obtained.

60
Question 9 What is the most likely bacterial
cause of her urinary tract infection?
  1. Proteus mirabilis
  2. E. coli
  3. Coagulase positive Staphlococus
  4. Alpha hemolytic Streptococcus
  5. Klebsiella pneumoniae

61
Bacteriology /Pathogenesis UTI - 1
  • Most Common - E. Coli, coliforms
  • Virulence Factors
  • adherence to uroepithelium by P-fimbriae
  • endotoxin release
  • Pyelo vs cystitis - 80 to 20

62
Bacteriology /Pathogenesis UTI 2
  • Perineal / urethral factors
  • uncircumcised - 10-20x risk
  • ? Urethral caliber (infant girls)
  • other myths such as bubble bath, wiping
    techniques
  • Low Urinary factors
  • dysfunctional voiding constipation
  • Other - indwelling catheters, congenital
    anomalies, Vesicoureteral reflux, sexual activity

63
Diagnosis
  • Leukocyte test and nitrite test
  • Urine culture gt 40-50,000 CFU/mL
  • Pyuria - not on recurrent UTIs

64
Clinical Issues
  • Lower tract - frequency, urgency, enuresis,
    dysuria
  • Upper tract - fever - nearly all in boys under 1
    year of age females peak in first year but still
    significant through the first decade
  • Asymptomatic bacteriuria - low risk

65
Radiological Evaluation
  • Renal ultrasound - anatomy, size, location,
    echogenicity
  • DMSA (2nd choice glucoheptanate - SGH) -
    cortical integrity, photopenic regions,
    differential function, abscess
  • CT scan - abscess
  • VCUG not the current standard for first UTI
    radionuclide for follow-up or siblings
  • IVP - NO WAY

66
AAP SEPT 2011Practice guidelines
  • DX Urinalysis with pyruria and culture with
    50,000 col/ml single organism by SPA or cath
  • Treat for 7-14 days
  • Ultrasound for all with febrile UTI
  • VCUG only with abnl Ultrasound, or other findings
    to suggest atypical or complex issues
  • VCUG after 2nd febrile UTI
  • No Abx prophylaxis unless VUR grade V

67
Grades of Reflux
68
Reflux Recommendationsthe simple way
  • GRADES I - III Antibiotics
  • GRADES IV - V Surgery
  • Although endoscopic approach is gaining favor
    over open reimplantation

69
Treatment
  • Oral
  • SMX-TMP, Amoxicillin/Clavulanate
  • Cefuroxime, cefprozil, cefixime, cefprodoxime
  • Parenteral
  • Neoates Ampicillin / Gentamicin
  • Older Children
  • Advanced level cephalosporin
  • Beta lactam beta lactamase inhibitor
  • Aminoglycoside ( ampicillin)

70
Case10 Case History
  • A 12 mo old girl is diagnosed with the first
    febrile UTI. She is not eating well. UA shows
    pyuria and bacteria. Urine culture is obtained
    and shows gt 100,000 colonies of E. Coli.
    Antibiotics are given.

71
Question 10 What is the most appropriate next
step?
  1. Perform a DMSA renal scan
  2. Refer to urology for cystoscopy
  3. Perform a renal sonogram and VCUG
  4. Perform urodynamics and flow studies
  5. Repeat urine culture in 3 months

72
Glomerulonephritis / Acute renal failure
73
Case 11
  • A 3 year old boy was attending summer camp. Five
    days later he presents with diarrhea, abdominal
    pain and appear pale. His mother finds out that
    there was cook out at camp. On examination the
    child is pale and is unable to void. His
    laboratory testing in your office shows a WBC of
    26,000, hemoglobin of 8 g/dL, platelets 98,000,
    Serum creatinine of 1 mg/dL, BUN 54 mg/dL,
    urinalysis with large blood, 100 mg/dL of protein.

74
Question 11 What is the most likely diagnosis?
  1. Henoch Schoenlein Purpura
  2. Post streptococcal glomerulonephritis
  3. IgA nephropathy
  4. Acute pyelonephritis
  5. Hemolytic uremic syndrome

75
Clinical prodrome
  • Diarrhea prodrome 1-15 days
  • Abdominal pain may be confused with ulcerative
    colitis, appendicitis, rectal prolapse,
    intussusception
  • Pallor
  • Irritability, restlessnes
  • Edema after rehydration
  • Oliguria/anuria

76
HUS Clinical manifestations
  • Thrombocytopenia
  • Hemolytic anemia
  • Renal failure
  • Neurologic (irritability, seizure, CVA)
  • Pancreatitis (IDDM) and colitis
  • Hypertension

77
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78
HUS Pathogenesis
  • Endothelial cell damage occurs secondary to toxin
    injury via binding to glycolipid receptor or
    lipopolysaccharide absorption.

79
HUS Differential diagnosis
  • Other forms of acute Glomerulonephritis / renal
    failure
  • Vasculitis
  • Urosepsis
  • Renal vein thrombosis
  • Coagulopathy (DIC)

80
Conservative management
  • Fluid restriction to ltinsensible losses plus
    urine output
  • Foley catheter limit to 24-48 hrs
  • Blood transfusion / platelets
  • Routine use of antibiotics controversial
  • Diuretics
  • Nutrition

81
Surgical Complications
  • Toxic megacolon
  • Rectal prolapse
  • Colonic gangrene
  • Intussusceptions
  • Perforation
  • Strictures
  • Mimic appendicitis, IBD

82
BONUS 1
  • John is a very dedicated weight lifter and
    started to take a creatine supplement. He asks
    you to explain the physiologic effects of
    creatine on his body. Which is the best
    explanations about creatine?
  • Creatine which is synthesized in the liver and
    kidney, is transported through the blood to be
    taken up by high energy demand of the brain and
    skeletal muscle.
  • Creatine supplementation affects hydration status
    and heat tolerance during exercise leading to
    muscle cramping and diarrhea
  • Creatine supplementation does improve
    physiological response to resistance exercise,
    increasing the maximal force production of
    muscles in men, not women.
  • Pasteurized cow's milk contains lower levels of
    creatine than human milk
  • Creatine has been shown to improve cognitive
    performance in young athletes.

83
BONUS 2
  • A 6 year old boy presents with recurrent episodes
    of brownish urine that developed coincidental
    with URIs. His urinalysis during the episodes
    reveals red blood cell casts and small protein.
    His complement levels are normal. His hepatitis
    B screen is normal. The MOST likely diagnosis
    is
  • Post Strep AGN
  • IgA nephropathy
  • Membranoproliferative glomerulonephritis
  • Membranous nephropathy
  • Wegeners granulomatosis

84
SOME QUICKIES
  • Unilateral multicystic dysplastic kidney MOST
    COMMOM CAUSE OF ABDOMINAL MASS IN NEWBORN
  • ULTRASOUND AND VCUG to DETERMINE CONTRALATERAL
    DISEASE
  • Polycystic Kidney Disease
  • Autosomal recessive bilateral enlarged kidneys
    with microcysts (Potters pulmonary
    hypoplasia) overtime liver fibrosis and
    failure
  • Autosomal dominant MOST COMMON INHERITED KIDNEY
    DISEASE bilateral enlarged kidneys with
    MACROCYSTS association with cerebral aneurysm
  • Hydronephrosis
  • Usually obstruction Ultrasound may show enlarged
    kidney without hydroureter

85
  • Posterior urethral valves
  • MOST COMMON CAUSE of OBSTRUCTIVE UROPATHY IN
    MALES remember poor urinary stream and palpable
    bladder
  • Vesicoureteral reflux
  • Grades from I to V VCUG diagnoses REFLUX,
    Radionuclide (DMSA) scans detect scars
    Surgery for grades IV to V (in general)
  • UTIs
  • E. coli most common followed by Klebsiella,
    Proteus. Males over 1 also have E. Coli then
    Proteus, Staph
  • Females gt males greater in uncircumcised
    remember constipation may increase incidence of
    UTIs
  • DX culture, cath or mid stream UA positive
    nitrites and leukocytes
  • 1st febrile UTI ultrasound VUCG for girls
    with more than 2 UTI in 6 months all males

86
  • Hematuria
  • Rule out hemoglobin and myoglobin
  • Brown urine glomerular look for red cell
    casts
  • Lower tract gross red (possible at end of
    stream), no casts, possible clots
  • Rule out hypercalciuria spot ratio
  • Common diseases
  • IgA gross hematuria with respiratory or GI
    illness
  • Post Strep AGN follows throat or skin infection
    by 10-21 days, Low C3 but recovers by 8-12
    weeks, maybe increased ASO titer
  • HUS E. coli 0157H7 MOST COMMON CAUSE OF
    ACUTE RENAL FAILURE low platelet count and
    microangiopathic hemolytic anemia (schistocytes
    coombs negative) cause undercooked meat or
    unpasteurized milk

87
  • Proteinuria
  • Rule out Orthostatic Proteinuria urine protein
    to creatinine ratio abnormal above 0.2 from AM
    specimen
  • Nephrotic syndrome ratio greater than 2 or more
    the 40 mg/m2/hr of protein
  • Minimal change steroid responsive is MOST
    COMMON
  • Usually 2- 6 yrs with mean of 4 yrs remember
    edema is dependent - first eyes than later in
    day to the legs.
  • Treatment prednisone 60 mg/m2/day for 4-6
    weeks followed by alternate day steroids for 4-6
    weeks.

88
  • Tubular entities
  • Diabetes insipidus
  • X linked or secondary to ADH resistance
  • Look for dilute urine in face of hypernatremia
  • Water deprivation test then give IV or intranasal
    vasopressin
  • Renal tubular acidosis normal anion gap
    hyperchloremic metabolic acidosis
  • Proximal or Type II bicarbonate wasting
  • Failure to thrive
  • pH lt 5.5
  • Serum bicarbonate usually less than 18
  • Remember FANCONI syndrome is RTA glycosuria,
    phosphaturia, and amino aciduria.
  • Treated with bicarbonate

89
  • Distal or Type I impaired distal acidification
    pH gt 5.5 may have hypokalemia and hypercalciuria
  • Look for polyuria, vomiting, nephrolithiasis
  • Treated with bicarbonate low dose compared to
    proximal
  • Bartter syndrome
  • Hypochloremic metabolic ALKALOSIS hypokalemia.
  • Look for polyuria, failure to thrive, low serum
    chloride and low potassium
  • Kidney Stones
  • Imaging of choice spinal CT.
  • Most stones are radiopaque since they contain
    calcium. Non-radiopaque are uric acid stones.
  • Cystinuria increased urinary excretion of
    dibasic amino acids

90
  • Acute kidney injury (failure)
  • Evaluation to determine prerenal (perfusion)
    renal (intrinsic) or post renal (obstruction)
  • Chronic kidney disease
  • Key items anemia, growth failure, renal
    osteodystrophy
  • Options dialysis and preferred renal
    transplantation

91
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92
Outline Part 2
  • Acute renal failure
  • Chronic renal failure
  • More Fluids Electrolytes
  • Tubular disorders
  • Cystic kidney disease

93
SCENARIO A 6 year boy is diagnosed as having ALL.
He is started on chemotherapy and his white blood
cell count drops precipitously. The child is
discharged and the family is encouraged. However,
after two days at home he spikes a temperature to
39 ?C. The parents contact the heme/ onc fellow
who tells them to come to the hospital
immediately. On arrival to the ER, the child is
a bit lethargic. His BP is 60/40. What is the
most important first step in the management of
this child? What are the most useful diagnostic
tests? What are the possible causes of his
condition? How should his condition be treated?
94
Acute Renal Failure (ARF) vs Pre-renal Azotemia
  • Key maneuver is restore RBF to distinguish
    reversible pre-renal state from short-term
    irreversible
  • Options
  • Bolus infusion of crystalloid solutions
  • Infusion of albumin
  • Administration of pressors
  • Administration of antagonists of clinical
    condition as in anaphylaxis

95
ARF Diagnosis
Pre-renal AGN ATN Obstruction
UA Marginal value Key RBC casts RTEC Marginal value
SG gt1.020 gt1.020 1.008-1.012 1.008-1.012
UNa lt20 lt20 gt40 gt40
FENA lt1 lt1 gt1 gt1
Uosm gt400 gt400 200-400 200-400
96
ARF Diagnosis
  • AGN
  • PSAGN
  • HSP
  • SLE
  • MPGN
  • Wegeners

97
ARF Diagnosis
  • ATN
  • Unreversed pre-renal azotemia
  • Nephrotoxic meds
  • Contrast agents
  • High calcium, uric acid, phosphate
  • Rhabdomyolysis (myoglobin)
  • Intravascular hemolysis (hemoglobin)

98
ARF Diagnosis
  • Obstructive uropathy
  • PUV
  • Prune belly
  • Vesicoureteric reflux
  • Neurogenic bladder (myelomeningocele)
  • Megacystis/megaureter
  • Secondary stones, fibrosis
  • Effect of age and gender

99
ARF Testing
  • Key labs BUN, creatinine, K
  • EKG
  • CXRay
  • Renal ultrasound
  • Specific blood tests based on underlying
    condition

100
ARF Management
  • Urgent issues
  • Potassium
  • Calcium
  • Glucose/insulin
  • NOT bicarbonate
  • Blood pressure parenteral therapy
  • Labetalol
  • Nitroprusside
  • ECF volume

101
ARF Conservative Management
  • Potassium
  • Diet restriction
  • Kayexalate
  • Blood pressure
  • IV/PO meds
  • ECF volume
  • Na restriction
  • Diuretic use need for furosemide

102
ARF Indications for Dialysis
  • Refractory hyperkalemia
  • Refractory hypertension
  • Symptomatic ECF volume overload
  • Symptomatic azotemia
  • Infection
  • Bleeding
  • CNS changes

103
ARF Pearls
  • Pre-renal azotemia and AGN are similar
  • ATN and post-renal failure are similar
  • Potassium kills first in ARF

104
SCENARIO A 6 year boy is seen at a routine
physical examination. Although he has no specific
complaints, his mother says he has been very
listless and his appetite is very poor. He has
not been playing well with his friends in play
group. Although he is toilet trained he seems to
be having more accidents during the night. On
examination, he looks a bit pale and tired. His
height has fallen from the 50 at his last visit
18 months ago to 10. His BP is 106/62 mm
Hg. What is the most important first step in the
diagnosing this childs problems? What are the
likely causes his condition? How should his
condition be treated?
105
CKD Diagnosis
  • Stages
  • CKD I renal injury GFR gt90
  • CKD II GFR 60-90
  • CKD III GFR 30-60
  • CKD IVGFR 15-30
  • CKD V ESRD

106
CKD Common features
  • Impact on growth
  • Impact on bone osteodystrophy
  • Impact on puberty
  • Impact on development social and cognitive

107
CKD Causes
  • Non-glomerular
  • Hypoplasia/dysplasia
  • Reflux nephropathy
  • Obstructive uropathy
  • PUV
  • Prune Belly
  • Neurogenic bladder

108
CKD Clinical manifestations
  • Growth failure
  • Dependent on age of onset
  • Dependent on level of GFR
  • UTIs
  • Pyelonephritis
  • Electrolyte abnormalities
  • Pseudohypoaldosteronism
  • Nephrogenic DI
  • Neurocognitive disability

109
CKD Diagnosis
  • Structural assessment
  • Imaging studies
  • US
  • VCUG dye vs radioisotope
  • DMSA scan
  • Retrograde studies, etc

110
CKD Diagnosis
111
SCENARIO A 15 year old girl comes to the clinic
because she has not had her period for the last 8
months. She feels tired all the time at home
school and is having a hard time concentrating in
school. She is not taking any medications
except for occasional NSAIDs for headaches and
some vitamins. Her parents are in good health.
On examination, her height and weight are
normal. Her BP is 162/98 mm Hg. She is pale and
has a mild amount of edema in both legs. She has
no rash or arthritis. What is the most important
first step in diagnosing this adolescents
problem? What are the most likely causes? How
should her condition be treated?
112
CKD Causes
  • Glomerular
  • FSGS
  • HUS
  • SLE
  • Membranoproliferative MPGN)
  • Alport
  • IgA Nephropathy
  • Membranous nephropathy
  • NOT diabetic or hypertensive nephropathy

113
CKD Clinical manifestations
  • Growth failure
  • Dependent on age of onset
  • Hypertension
  • Role of ?ECF volume and ?PRA
  • Electrolyte abnormalities
  • Acute
  • Hyperkalemia
  • Edema
  • Signs of underlying disease

114
CKD Diagnosis
  • Low value of radiology tests
  • Blood tests
  • C3, C4, CH50
  • ASLO
  • ANA, dsDNA, Ro, La, Sm
  • ANCA
  • Anti-GBM
  • Renal biopsy

115
CRF Management
  • Nutritional supplementations
  • CHO deficiency
  • Protein restriction
  • Impact on growth
  • Effect in more advanced CKD
  • BP control
  • Disease progression
  • ACEI/ARB

116
CRF Management
  • Interference with renin-angiotensin aldosterone
    axis
  • Safety of ACEI even with advanced CKD
  • Role of combined ACEI/ARB
  • Effect of aldosterone antagonists
  • Safety issues
  • Hyperkalemia
  • Reduction in GFR

117
CRF Management
  • Endocrine treatments
  • rhGH
  • Doubles growth velocity
  • Minimal risk of progression
  • Erythropoietin
  • Nearly always effective
  • Antibody induced pure red cell aplasia
  • Calcitriol
  • IV route
  • More selective agents

118
CRF Pearls
  • Chronic glomerular diseases have oliguria vs
    chronic tubular diseases which can have polyuria
    and sodium loss
  • Nocturia and enuresis may indicate CRF
  • Severity of growth failure and neurocognitive
    deficits are inversely related to age of onset of
    CRF

119
CRF More pearls
  • Most important feature of nutritional support is
    to correct low caloric intake
  • Medication doses need to be adjusted as GFR
    declines
  • Almost no form of CRF is a contraindication to
    transplant

120
SCENARIO A 10-day male infant presents with a
history of irritability, low grade fever, emesis
and diarrhea. Prenatal and family history is
non-contributory. On examination the infant is
irritable, temp is 38C, has mottled skin and a
capillary refill of 4 sec. The systolic blood
pressure is barely palpable and the pulse is 195
beats/min. The anterior fontanelle is flat.
Hemoglobin 18 g/dl White cell
count 30,000 Platelets 280,000 What are key
features in the history and examination? What
studies would you perform? What is your initial
therapy? What is your initial diagnosis (es)?
121
Electrolyte Disorders Sodium
  • KEY function of Na
  • ECF cation
  • Maintenance of intravascular compartment
  • Disturbances in ECF volume are secondary to
    disturbances in Na balance
  • ECF volume assessment is clinical
  • Reduced see dehydration above
  • Increased pulmonary and/or peripheral edema

122
Electrolyte disorders Sodium
123
Electrolyte Disorders Sodium
  • History
  • Source of Na loss
  • Change in body weight
  • Renal response to low ECGF volume
  • Oliguria
  • Reduced urine Na
  • Reduced FENA

124
Electrolyte disorders Sodium
125
Electrolyte disorders Sodium
  • Hypernatremia
  • Risk factors
  • Breast feeding
  • Feeding errors
  • Impaired thirst
  • Impaired access to water
  • Presentation
  • Irritability, seizures
  • Treatment
  • SLOW
  • HYPOTONIC FLUIDS 1/5 NS

126
Electrolyte disorders Sodium
  • Hyponatremia
  • Risk factors
  • Feeding errors (Keating)
  • Salmonella diarrhea
  • Increased extra-renal salt loss
  • Pain, anesthesia, post-operative picture
  • Female gender
  • Presentation
  • Lethargy, seizures
  • Treatment
  • ?SLOW
  • Correction ?25 mmol/L OR ?130 mmol/L over
    initial 48 hr

127
Electrolyte disorders Sodium
  • Bad outcomes
  • Brain
  • Hemorrhage and cerebral edema in hypernatremia
  • Osmotic demyelinating syndrome and acute CNS
    deterioration in Hyponatremia
  • DKA
  • ?Hyponatremia (?100 glucose mg/dl ? ?1.6 Na
    meq/l)
  • Comparison to hypernatremia

128
SCENARIO A 4-week old infant presents with a
history of irritability, low grade fever and poor
feeding. Prenatal and family history is
non-contributory. On examination the infant is
irritable, temp is 37C, has dark skin and a
capillary refill of 4 sec. The systolic blood
pressure is barely palpable and the pulse is 195
beats/min. The anterior fontanelle is sunken.
Hemoglobin 18 g/dl White cell
count 30,000 Platelets 280,000 What are key
features in the history and examination? What
studies would you perform? What is your initial
therapy? What is your initial diagnosis (es)?
129
Electrolyte Disorders Potassium
  • KEY function of K
  • ICF cation
  • Transmembrane potential, secretion,
    neuromechanical coupling
  • Disturbances in K reflect sudden changes in
    serum concentration and transmembrane ratio
  • Assessment is linked to cardiac impact of
    abnormal K concentration

130
Electrolyte disorders Potassium
  • Regulatory organs
  • Kidney secretion
  • Na
  • Urine flow rate
  • Adrenal
  • Aldosterone
  • GI tract
  • Transmembrane
  • pH
  • Osmolality
  • Beta adrenergics
  • Insulin
  • Diet

131
Potassium
  • Key tests
  • BUN, Cr, Na, K, bicarbonate
  • Urine K useless
  • Urine Na/K ratio
  • Hormones
  • PRA
  • Aldosterone

132
Electrolyte disorders Potassium
  • Hyperkalemia
  • EKG
  • Peaked T waves
  • Treatment
  • Calcium infusion
  • Glucose/insulin
  • NOT Bicarbonate
  • Kayexalate
  • DIALYSIS

133
Hyperkalemia differential diagnosis
  • No real disease
  • Increase cells WBC, polycythemia,
    thrombocytosis, crush injury
  • Transmembrane
  • Renal
  • ARF
  • CRF
  • Liddles
  • Adrenal
  • Adrenal failure
  • Congenital adrenal hyperplasia ambiguous
    genitalia
  • Isolated renin abnormalities

134
Hyperkalemia Work-up
  • BUN, creatinine, Na, K, Bicarbonate
  • PRA
  • Aldosterone
  • Urinary Na/K ratio

135
Electrolyte disorders Potassium
  • Hypokalemia
  • EKG
  • U waves
  • Treatment
  • Restore ECF volume to ?2?hyperaldosteronism
  • PO potassium
  • Limitations tolerance
  • IV potassium
  • Limitation 0.3 meq/kg/hr
  • Central vs peripheral IV

136
Hyperkalemia differential diagnosis
  • Systemic
  • Malnutrition
  • Adrenal
  • Adrenal overactivity
  • Congenital adrenal hyperplasia
  • Primary renin abnormalities
  • Renal
  • DKA
  • Osmotic diuresis

137
SCENARIO A 15 month child presents with a history
of poor feeding and impaired growth. Prenatal and
family history is non-contributory. On
examination the infants height and weight are
below the 5th percentile. The systolic blood
pressure is 102 and the pulse is 110. The rest of
the examination is normal. Na 138 Cl 114 Bicarb
onate 16 What are key features in the history
and examination? What studies would you
perform? What is your initial therapy? What is
your initial diagnosis (es)?
138
Electrolyte disorders acid-base
139
Electrolyte disorders
  • Anion gap
  • Na Cl HCO3
  • Normal value 4-12
  • Impact of serum albumin

140
Electrolyte disturbances RTA
  • Metabolic acidosis
  • Normal anion gap -- hyperchloremic
  • Diarrhea
  • RTA
  • High anion gap -- normochloremic
  • MUDPIES or KUSSMAUL
  • Key entities
  • DKA
  • Lactic acidosis
  • Uremia
  • Metabolic disease
  • Toxins

141
Electrolyte disturbances RTA
  • Proximal
  • Low K
  • Primary
  • Secondary
  • Glycogen storage
  • Wilsons, fructose intolerance, tyrosinemia
  • ?PTH, ?Vitamin D
  • Cystinosis

142
Electrolyte disturbances RTA
  • Distal
  • Primary
  • Secondary
  • Transplant rejection
  • Drugs amphotericin, cisplatinum
  • Collagen vascular disease

143
Electrolyte disorders RTA
  • Assessment
  • SMAC Cl-
  • VBG Bicarbonate
  • Urine calcium, citrate
  • Urine anion gap unmeasured cation (NH4)
  • Xrays

144
Electrolyte disturbances RTA
  • Treatment
  • Proximal
  • Higher doses of bicarbonate
  • More frequent dosing
  • Exacerbation of hypokalemia with Rx
  • Distal
  • 1-3 mmol/kg varying with age and diet
  • 3 doses
  • Stabilization of K with Rx

145
Electrolyte disorders Fanconis
146
Electrolyte disorders metabolic alkalosis
  • Extrarenal/GI loss of K
  • CF
  • Vomiting
  • NG suction
  • Pyloric stenosis
  • Distal GI loss of bicarbonate
  • Chloride diarrhea
  • Renal
  • Bartters
  • Gitelmans
  • Apparent mineralocorticoid excess (AME)/licorice

147
Electrolyte disorders DI
  • Central
  • Nephrogenic
  • Risk of CNS disease
  • 1/12 (1/3 X ¼) of loss from ECF
  • Limited access to water
  • Altered thirst

148
Electrolyte disorders DI
  • Central
  • AVP replacement
  • Nephrogenic
  • Adequate water intake
  • Low solute diet
  • Hydrochlorothiazide

149
Electrolytes Pearls
  • There are three pure renal causes of FTT
    azotemia, DI, and RTA
  • RTA causes hyperchloremic acidosis
  • Bartters and Gitelmans differ in calcium
    excretion high in former low in latter

150
Thank you
  • GOOD LUCK
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