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Metropolitan New York / New Jersey Pediatric Board Review Course Pediatric Nephrology May, 2008

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Title: Metropolitan New York / New Jersey Pediatric Board Review Course Pediatric Nephrology May, 2008


1
Metropolitan New York / New Jersey Pediatric
Board Review Course Pediatric Nephrology May,
2008
  • Leonard G. Feld MD PhD
  • Levine Childrens Hospital
  • Charlotte, NC
  • Howard Trachtman MD
  • Schneider Childrens Hospital
  • New Hyde Park, NY

2
Materials
  • Consider all other material to help you achieve a
    passing score
  • American Academy of Pediatrics Pediatrics
    Review and Education Program (PREP), Pediatrics
    in Review.

3
Renal / Urology
  • General
  • Normal function
  • Proteinuria
  • Hematuria
  • Persistent microscopic hematuria
  • Causes of gross and microscopic hematuria
  • Dysuria / Incontinence
  • Congenital
  • Renal dysplasia
  • Abnormalities of the collecting system, kidney,
    bladder

4
Renal / Urology
  • Acquired
  • Infection of the urinary tract
  • Acute glomerulonephritis
  • Nephrotic syndrome
  • Hemolytic uremic syndrome
  • Henoch-Schoenlein purpura
  • IgA nephropathy
  • Other
  • Renal Failure,Trauma, renal stones, RTA

5
Renal / Urology
  • Hypertension
  • Nephrogenic diabetes insipidus
  • Cystinosis

6
Outline Part 1
  • Hematuria
  • Proteinuria
  • Hypertension
  • Urinary tract infections
  • Glomerulonephritis

7
A 3-week-old 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 of of 39.0C, has mottled skin
and a capillary refill of 4 sec. The systolic
blood pressure is normal and the pulse is 185
beats/min. The anterior fontanelle is full.
Hemoglobin 14 g/dl White cell
count 30,000 Platelets 110,000 What studies
would you like to perform. What is your initial
therapy? What is your initial diagnosis (es)?
8
Answers
  • Blood culture, urine, CXR, and LP
  • Fluid resuscitation broad spectrum antibiotics
  • Late onset neonatal sepsis / meningitis

9
From Consensus in Pediatrics Fever In Infants and
Children, Feld LG, Hyams J eds. Mead Johnson
Nutritionals, 2007.
10
PEARL No question on the lt 28 day old febrile
infant
11
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.
  • What is the next step? What would be a major
    consideration for a referral to a pediatric
    nephrologist?

12
  • 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.
  • Caveat - Family anxiety because of the
    connotation of blood and cancer in adults.

13
Classification of Hematuria
  • Microscopic (vast majority of the cases)
  • Transient
  • Persistent
  • Macroscopic (urologic / renal disorders)
  • Transient
  • Persistent (gt 2 weeks)
  • Persistent microscopic/ Transient macroscopic
  • IgA or Bergers benign recurrent hematuria

14
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

15
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

16
Initial evaluation of the patient with hematuria
  • All patients BUN, creatinine, CBC, kidney and
    bladder ultrasound
  • Probable glomerular hematuria
  • C3, ASO titer
  • possible hepatitis, HIV, SLE serology
  • renal biopsy
  • Probable non-glomeurlar hematuria
  • urine culture, urine Ca/creatinine ratio
  • possible hemoglobin electrophoresis,
  • coagulation studies, isotope scans,
  • Flat plate, CT, ??IVP, cystoscopy

17
Pearls for Hematuria
  • Hematuria is 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.

18
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.

19
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 is hypercalciuria, SS trait, or thin
    basement membrane disease.
  • Cysto is rarely helpful

20
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 with a BP of
95/65, no edema, some suprapubic tenderness and
red tympanic membranes. The mother thinks that a
similar episode occur on vacation a few months
ago. WHAT WOULD YOU LIKE TO DO?
21
Tests
  • Normal electrolytes
  • Creatinine 0.5 mg/dl
  • Urinalysis large blood, no protein
  • Urine culture no growth

22
More to the story
  • She calls with a recurrent episode of gross
    hematuria with a URI three months later
  • So what do you do ?

23
Other tests
  • ANA, ANCA, ASO, Family screening
  • Complement C3NF

24
Now what
  • 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

25
(No Transcript)
26
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 - - - -
27
  • 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.
  • a. Urinalysis
  • b. Blood tests for total protein and albumin
  • c. Serum creatinine
  • d. Sedimentation rate
  • e. Serum complement (C3)

28
  • 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 intravenous pyelogram
  • Obtain a voiding cystourethrogram
  • Perform a CBC in the office
  • Order an ASO titer

29
  • An 8-year-old boy presents with tea colored
    urine. He has very mild edema. The work-up should
    include all the following except.
  • Complement studies
  • Serum creatinine
  • Urinalysis for protein
  • Monitor blood pressure and urine output
  • Obtain an intravenous pyelogram and VCUG

30
Proteinuria
  • John is an 12 year old noted on a basketball team
    physical to have 2 protein on dipstick. He has
    an unremarkable past medical history. Family
    history is negative in the parents and siblings
    for any renal disease. A repeat urine in one
    week in his PMDs office is still positive.
  • What is the next step? Should you refer?

31
  • 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.
  • Caveat - Family anxiety because of the
    connotation of protein and friends told them
    about kidney failure.

32
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

33
What does Orthostatic Proteinuria mean?
Protein Excretion
34
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

35
Hypertension
36
Hypertension
  • Case David is a 10 year old boy first noted to
    have an elevated blood pressure of 140/85 during
    a PE for headaches. Pt has a long history of
    learning and behavioral issues. Headache
    evaluation was normal (CT, sinus,etc.). Referred
    for evaluation. Initial evaluation noted a Ht /
    Wt gt 99tile, BP of 128/86 mmHg, normal
    ultrasound and renal scan, although a plasma
    renin of 8 ng/ml/min (nl lt 2).
  • Do you perform an angiogram?

37
Definition of Hypertension The 4th Report on
High Blood Pressure in Children and Adolescents
  • Hypertensionaverage SBP and/or DBP that is
    greater than or equal to the 95th percentile for
    sex, age, and height on 3 or more occasions.
  • Prehypertensionaverage SBP or DBP levels that
    are greater than or equal to the 90th percentile,
    but less than the 95th percentile.
  • Adolescents with BP levels greater than or equal
    to 120/80 mmHg should be considered
    prehypertensive.

38
Evaluation of Hypertension
39
Therapeutic Lifestyle Changes
  • Normal Encourage healthy diet, sleep, and
    physical activity.
  • Prehypertension Recommend weight management
    counseling if overweight introduce physical
    activity and diet management.
  • Stage 1 hypertension Recommend weight management
    counseling if overweight introduce physical
    activity and diet management.
  • Stage 2 hypertension Recommend weight management
    counseling if overweight introduce physical
    activity and diet management.

40
Indications for Treatment
  • Symptomatic hypertension
  • Secondary hypertension
  • Hypertensive target-organ damage
  • Diabetes (types 1 and 2)
  • Persistent hypertension despite nonpharmacologic
    measures

41
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.

42
Urinary Tract Infections
43
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.
    Antibiotics are given (SMX-TMP).
  • How to proceed?
  • What are some of your concerns?
  • Radiographic follow-up
  • Long-term monitoring

44
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

45
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

46
Diagnosis
  • Leukocyte test and nitrate test
  • Urine culture gt 40-50,000 CFU/mL
  • Pyuria - not on recurrent UTIs

47
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

48
Radiological Evaluation
  • Renal ultrasound - anatomy, size, location,
    echogenicity
  • DMSA (2nd choice glucoheptanate - SGH) -
    cortical integrity, photopenic regions,
    differential function, abscess
  • CT scan - abscess
  • VCUG - standard for first UTI radionuclide for
    follow-up or siblings
  • IVP - NO WAY

49
Grades of Reflux
50
Reflux Recommendations the simple way
  • GRADES I - III Antibiotics
  • GRADES IV - V Surgery

51
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)

52
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.
    Antibiotics are given (SMX-TMP).
  • How to proceed?
  • What are some of your concerns?
  • Radiographic follow-up
  • Long-term monitoring

53
The Suggested Answers
  • What are your concerns?
  • Voiding history
  • Radiographic studies
  • ultrasound and VCUG
  • Follow-up (no reflux)
  • cultures every month for three months, then every
    other month for six months
  • ( every 4 months)
  • Follow-up (reflux) - antibiotics

54
Glomerulonephritis / Acute renal failure
55
Case History
  • 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 - How to
    proceed?
  • What are some of your concerns?

56
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

57
HUS Clinical manifestations
  • Thrombocytopenia
  • Hemolytic anemia
  • Renal failure
  • Neurologic (irritability, seizure, CVA)
  • Pancreatitis (IDDM) and colitis
  • Hypertension

58
HUS Pathogenesis
  • Endothelial cell damage occurs secondary to toxin
    injury via binding to glycolipid receptor or
    lipopolysaccharide absorption.

59
HUS Differential diagnosis
  • Other forms of acute Glomerulonephritis / renal
    failure
  • Vasculitis
  • Urosepsis
  • Renal vein thrombosis
  • Coagulopathy (DIC)

60
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

61
Surgical Complications
  • Toxic megacolon
  • Rectal prolapse
  • Colonic gangrene
  • Intussusceptions
  • Perforation
  • Strictures
  • Mimic appendicitis, IBD

62
Case
  • A four year old boy presents with a three day
    history of periorbital swelling and sox
    indentations around his ankles. He has been
    healthy without any intercurrent illnesses. The
    family and past medical history are unremarkable.
    On examination he has pretibial edema and has
    gained 2.5 kg since his examination 2 months ago
    for an otitis media.
  • What are your thinking?

63
Nephrotic Syndrome
64
Definition
  • Nephrotic syndrome is a clinical state
    characterized by heavy proteinuria and
    hypoalbuminemia, often associated with edema,
    hypercholesterolemia, and generalized
    hyperlipidemia.

65
PRIMARY NEPHROTIC SYNDROME
  • 90 childhood cases
  • unassociated with systemic disease

66
CLINICAL PRESENTATION (1)
  • EDEMA is the major symptom - first periorbital,
    then generalized. Happy parents finally my
    child is gaining weight.
  • Soft and pitting in nature.
  • May cause anasarca with ascites, pleural
    effusions, labial and scrotal swelling.

67
CLINICAL PRESENTATION (2)
  • Poor appetite
  • Diarrhea during massive edema
  • Hepatomegaly
  • Abdominal pain (need to r/o peritonitis or
    surgical abdomen)
  • Respiratory difficulty
  • Hypertension (15-20 of MCNS)

68
LABORATORY FEATURES
  • PROTEINURIA is the primary abnormality.
  • Selective- almost entirely albuminuria
  • gt 40 mg/m2/hr or
  • U protein/creatinine ratio gt 1 (mg/mg)
  • due to loss of charge selectivity of glomerular
    basement membrane

69
COMPLICATIONS OF NEPHROTIC SYNDROME
70
INFECTION
  • Impaired resistance to infection
  • -low immunoglobulin levels
  • -generalized protein deficiency
  • -defective opsonization
  • -splenic hypofunction
  • immunosuppressive therapy
  • Peritonitis
  • Pneumococcal infection

71
TREATMENT
  • Diuretics ?
  • Albumin Lasix infusion
  • Prednisone 60 mg/m2/d x 4 weeks, then 40 mg/m2
    every other day x 4 wk
  • Alternatives
  • Cyclophosphamide 2-3 mg/kg/d x 8 weeks, not to
    exceed 200 mg/kg
  • Chlorambucil
  • Cyclosporine

72
Requirements at a glance Nephrolithiasis
  • Presentation
  • Most patients present with abdominal, flank or
    pelvic pain depending upon the location of the
    calculus. Referred pain may be localized to the
    scrotum, penis or female genitalia.
  • Patients may have associated nausea and vomiting,
    gross hematuria, or symptoms of a urinary tract
    infection (urinary frequency, dysuria, etc.)
  • Not all patient with urinary calculi have gross
    or microscopic hematuria
  • 20 of patients with microscopic hematuria and
    hypercalciuria will develop a urinary calculus
    within five years.

73
Requirements at a glance Nephrolithiasis
  • Medical Evaluation
  • Family history is paramount as pediatric stones
    may be associated with inherited disorders such
    as cystinuria, primary hyperoxaluria or renal
    tubular acidosis.
  • Patients past medical history including low
    fluid intake, dietary exess or deficiencies may
    predispose to calcium oxalate stone formation.
  • Patients with a history of hyperthyroidism,
    myeloproliferative disorders, gastrointestinal
    disorders, chronic urinary tract infections or
    immobilization may be at increased risk for
    stone formation.
  • Infants with a history of furosamide (Lasix) use
    are at an increased risk for stones and
    nephrocalcinosis.
  • Pediatric patients with a history of stones are
    at an increased risk for recurrent stone
    formation

74
Requirements at a glance Nephrolithiasis
  • Radiographic evaluation
  • CT scan without contrast is the most sensitive
    study for the detection of urinary calculi.
  • KUB and renal ultrasound may be useful in
    specific situations.
  • Laboratory Evaluation
  • Urinalysis of a first morning void including pH,
    specific gravity and present of bacteria are
    useful in the evaluation.
  • Measurement of serum electrolytes including
    sodium, potassium, bicarbonate, chloride, uric
    acid, calcium, phosphorus and creatinine may
    provide useful information.
  • A 24 hour urine analysis for volume, calcium,
    oxalate, citrate, uric acid, cystine, sodium,
    phosphate and creatinine. Measurements must be
    corrected to patient body mass.

75
Outline Part 2
  • Dehydration
  • Acute renal failure
  • Chronic renal failure
  • Fluids Electrolytes
  • Tubular disorders
  • Cystic kidney disease

76
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)?
77
Dehydration Clinical
  • Importance of clinical history
  • Feeding history (BF, formula error)
  • Other conditions (CF, CV disease)
  • Features to assess change in weight, altered VS,
    orthostatic changes. turgor/refill, fontanelle,
    tears
  • No specific diagnostic laboratory test only
    supportive
  • Key feature is reversibility

78
Dehydration Laboratory
  • Urine
  • S.G.
  • UNa, FENA, Uosm
  • Microscopic examination
  • Blood
  • BUN, creatinine
  • Bicarbonate

79
Dehydration Therapy
  • Emergent therapy
  • 10-20 ml/kg boluses
  • Isotonic solution
  • Repeat until any evidence of improvement
  • Correction therapy
  • Isotonic over 24 hours
  • Ongoing losses diarrhea, vomiting
  • Maintenance therapy

80
Dehydration Pearls
  • Dehydration is reversible
  • Dehydration is a misnomer and reflects loss of
    sodium not water
  • Isotonic solutions are fluid of choice
  • FENA is best test to assess severity of
    dehydration

81
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?
82
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

83
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
84
ARF Diagnosis
  • AGN
  • PSAGN
  • HSP
  • SLE
  • MPGN
  • Wegeners

85
ARF Diagnosis
  • ATN
  • Unreversed pre-renal azotemia
  • Nephrotoxic meds
  • Contrast agents
  • High calcium, uric acid, phosphate
  • Rhabdomyolysis (myoglobin)
  • Intravascular hemolysis (hemoglobin)

86
ARF Diagnosis
  • Obstructive uropathy
  • PUV
  • Prune belly
  • Vesicoureteric reflux
  • Neurogenic bladder (myelomeningocele)
  • Megacystis/megaureter
  • Secondary stones, fibrosis
  • Effect of age and gender

87
ARF Testing
  • Key labs BUN, creatinine, K
  • EKG
  • CXRay
  • Renal ultrasound
  • Specific blood tests based on underlying
    condition

88
ARF Management
  • Urgent issues
  • Potassium
  • Calcium
  • Glucose/insulin
  • NOT bicarbonate
  • Blood pressure parenteral therapy
  • Labetalol
  • Nitroprusside
  • ECF volume

89
ARF Conservative Management
  • Potassium
  • Diet restriction
  • Kayexalate
  • Blood pressure
  • IV/PO meds
  • ECF volume
  • Na restriction
  • Diuretic use need for furosemide

90
ARF Indications for Dialysis
  • Refractory hyperkalemia
  • Refractory hypertension
  • Symptomatic ECF volume overload
  • Symptomatic azotemia
  • Infection
  • Bleeding
  • CNS changes

91
ARF Pearls
  • Pre-renal azotemia and AGN are similar
  • ATN and post-renal failure are similar
  • Potassium kills first in ARF

92
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?
93
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

94
CKD Common features
  • Impact on growth
  • Impact on bone osteodystrophy
  • Impact on puberty
  • Impact on development social and cognitive

95
CKD Causes
  • Non-glomerular
  • Hypoplasia/dysplasia
  • Reflux nephropathy
  • Obstructive uropathy
  • PUV
  • Prune Belly
  • Neurogenic bladder

96
CKD Clinical manifestations
  • Growth failure
  • Dependent on age of onset
  • Dependent on level of GFR
  • UTIs
  • Pyelonephritis
  • Electrolyte abnormalities
  • Pseudohypoaldosteronism
  • Nephrogenic DI
  • Neurocognitive disability

97
CKD Diagnosis
  • Structural assessment
  • Imaging studies
  • US
  • VCUG dye vs radioisotope
  • DMSA scan
  • Retrograde studies, etc

98
CKD Diagnosis
99
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?
100
CKD Causes
  • Glomerular
  • FSGS
  • HUS
  • SLE
  • Membranoproliferative MPGN)
  • Alport
  • IgA Nephropathy
  • Membranous nephropathy
  • NOT diabetic or hypertensive nephropathy

101
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

102
CKD Diagnosis
  • Low value of radiology tests
  • Blood tests
  • C3, C4, CH50
  • ASLO
  • ANA, dsDNA, Ro, La, Sm
  • ANCA
  • Anti-GBM
  • Renal biopsy

103
CRF Management
  • Nutritional supplementations
  • CHO deficiency
  • Protein restriction
  • Impact on growth
  • Effect in more advanced CKD
  • BP control
  • Disease progression
  • ACEI/ARB

104
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

105
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

106
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

107
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

108
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)?
109
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

110
Electrolyte disorders Sodium
111
Electrolyte Disorders Sodium
  • History
  • Source of Na loss
  • Change in body weight
  • Renal response to low ECGF volume
  • Oliguria
  • Reduced urine Na
  • Reduced FENA

112
Electrolyte disorders Sodium
113
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

114
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

115
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

116
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)?
117
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

118
Electrolyte disorders Potassium
  • Regulatory organs
  • Kidney secretion
  • Na
  • Urine flow rate
  • Adrenal
  • Aldosterone
  • GI tract
  • Transmembrane
  • pH
  • Osmolality
  • Beta adrenergics
  • Insulin
  • Diet

119
Potassium
  • Key tests
  • BUN, Cr, Na, K, bicarbonate
  • Urine K useless
  • Urine Na/K ratio
  • Hormones
  • PRA
  • Aldosterone

120
Electrolyte disorders Potassium
  • Hyperkalemia
  • EKG
  • Peaked T waves
  • Treatment
  • Calcium infusion
  • Glucose/insulin
  • NOT Bicarbonate
  • Kayexalate
  • DIALYSIS

121
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

122
Hyperkalemia Work-up
  • BUN, creatinine, Na, K, Bicarbonate
  • PRA
  • Aldosterone
  • Urinary Na/K ratio

123
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

124
Hyperkalemia differential diagnosis
  • Systemic
  • Malnutrition
  • Adrenal
  • Adrenal overactivity
  • Congenital adrenal hyperplasia
  • Primary renin abnormalities
  • Renal
  • DKA
  • Osmotic diuresis

125
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)?
126
Electrolyte disorders acid-base
127
Electrolyte disorders
  • Anion gap
  • Na Cl HCO3
  • Normal value 4-12
  • Impact of serum albumin

128
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

129
Electrolyte disturbances RTA
  • Proximal
  • Low K
  • Primary
  • Secondary
  • Glycogen storage
  • Wilsons, fructose intolerance, tyrosinemia
  • ?PTH, ?Vitamin D
  • Cystinosis

130
Electrolyte disturbances RTA
  • Distal
  • Primary
  • Secondary
  • Transplant rejection
  • Drugs amphotericin, cisplatinum
  • Collagen vascular disease

131
Electrolyte disorders RTA
  • Assessment
  • SMAC Cl-
  • VBG Bicarbonate
  • Urine calcium, citrate
  • Urine anion gap unmeasured cation (NH4)
  • Xrays

132
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

133
Electrolyte disorders Fanconis
134
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

135
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

136
Electrolyte disorders DI
  • Central
  • AVP replacement
  • Nephrogenic
  • Adequate water intake
  • Low solute diet
  • Hydrochlorothiazide

137
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

138
Thank you
  • GOOD LUCK
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