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Metro New York / New Jersey Pediatric Board Review Course
Pediatric NephrologyJune 2013Leonard G. Feld MD PhD
Levine Children’s HospitalCharlotte, NC
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March 17, 2005
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For the ExamFluid and Electrolyte MetabolismA. 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
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For the ExamRenal DisordersA. Normal function, proteinuria, hematuria, persistent microscopic hematuria, causes of
gross and microscopic hematuria, nonhematogenous etiology of red urine,dysuria, B. Incontinence - Nocturnal, functional, daytime incontinence, C. Congenital - Renal dysplasia, unilateral multicystic dysplastic kidney, autosomal
dominant polycystic kidney disease, autosomal recessive polycystic kidney disease, Juvenile nephronophthisis, Renal agenesis
D. Abnormalities of the collecting system, kidney, and bladder – Hydronephrosis, Hydroureter and megaureter, Ureterocele, Vesicoureteral reflux,
E. Abnormalities of the urethra - Posterior urethral valves, Urethral strictureF. Hereditary nephropathy - Familial nephritis, Congenital nephrotic syndromeG. Acquired - Infection of the urinary tract (Pyelonephritis, Cystitis), acute
glomerulonephritis, Nephrotic syndrome, Hemolytic-uremic syndrome, Henoch-Schoenlein purpura,IgA nephropathy
H. Renal failure - Prerenal failure, Intrinsic renal failure , chronic renal failure, I. Other - Trauma, renal injuries, Urethral injury, Toxins, Renal stones, Renal tubular
acidosis, Hereditary conditions with renal manifestations, Nephrogenic diabetes, insipidus, Cystinosis
J. Hypertension – General, Renal, Vascular, Adrenal, Pheochromocytoma, Cushing syndrome, Miscellaneous causes, Essential hypertension, Administration of drugs, Traction on legs
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ANSWERS TO THE CONTENT SPECIFICATIONS
• Fluid and electrolyte
• Nephrology
SUBJECT LINE: NYNJ PEDS
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Outline• Fluid and Electrolyte abnormalities
– Dehydration
• Renal– Hematuria– Proteinuria– Hypertension– Urinary tract infections– Glomerulonephritis
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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.
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March 17, 2005
Electrolyte Composition of Body Fluid Compartments
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BODY WATER DISTRIBUTION
TOTAL BODY WATER (TBW)0.6 x Body Weight (BW)
EXTRACELLULAR FLUID (ECF)
0.2 x BW
INTRACELLULAR FLUID (ICF)0.4 x BW
Interstitial Fluid Plasma ¾ of ECF ¼ of ECF
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Clinical AssessmentFluid
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, < skin turgor, sunken eyes, deep resp, weak pulses, cool extremities, oliguria
Severe
(15%)
150 cc/kg Shock Intense thirst, BP, cap refill > 3 sec, weak pulses, apnea/rapid breathing, coma, anuria
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Maintenance RequirementsBody wt 0-10 kg 10-20 kg 20 kg
TBW 100 ml/kg 1000 ml +
50 ml/kg for each kg > 10kg
1500 +
20 ml/kg for each kg > 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
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Deficit
Type of Dehydrationbased 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] < 130 100-120 10-12 8-10
Hypernatremic [Na] > 150 100-120 2-4 0-4
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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%);
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Hyponatremia
• Serum [Na+] < 130 mEq/L
• Water shifts into cells – lower ECF volume
• <125 mEq/L – nausea and malaise
• < 120 mEq/L – headache, lethargy,
• <115 mEq/L – seizure and coma
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Loss of hypertonic Fluid and Sodium from the ECF secondary to Dehydration
35 Na 64 K
15 Na55 K
10 Na
15 Na54 K
10 Na
280
0
280
0
280
0
258
0
200
0
258
0
0.25 Liters 0.40 Liters
0.15 Liters 0.40 Liters
0.116 Liters 0.434 Liters
NORMAL
AFTER LOSS
AFTER OSMOTIC ADJUSTMENT
NORMAL ECF NORMAL ICF
mOsm/L
400
0
Loss0.10 LIters
10 K10 Na
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Question 1: What is the appropriate parenteral solution
A. 5% dextrose + 0.45% isotonic saline + 40 mEq KCl /L
B. 0.45% isotonic saline + 40 mEq KCl /L
C. 0.9% isotonic saline + 40 mEq KCl /L
D. 5% dextrose + 40 mEq KCl /L
E. 5% dextrose + 0.2% isotonic saline
5% dextrose
+ 0.45% isot..
.
0.45% isoto
nic sa
line + 4...
0.9% isoto
nic sa
line + 40...
5% dextrose
+ 40 mEq KCl /
L
5% dextrose
+ 0.2% isoto
n..
20% 20% 20%20%20%
6
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ApproachFor this 6.6 kg infant Maintenance requirements for 24 hours
Water 100 mL / kg x 6.6 kg = 660 mLSodium 3 mEq / 100 mL x 660 mL = 20 mEqPotassium 2 mEq / 100 mL x 660 mL = 13 mEq
For this 6 kg infant with hyponatremic dehydration at 10%Deficits for 24 hoursWater Pre-illness weight – Illness weight =
6.6 – 6 kg = 0.6 kg = 600 mLSodium 10 mEq x 6.6 kg = 66 mEqPotassium 8 mEq x 6.6 kg = 53 mEq
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Points on Hypernatremic Dehydration
35 Na 64 K
31 Na
62 K2 Na
37 Na62 K2 Na
280
0
280
0
280
0
318
0
0.25 Liters 0.40 Liters
0.15 Liters
0.40 Liters
0.195 Liters 0.355 Liters
NORMAL
AFTER LOSS
AFTER OSMOTIC ADJUSTMENT
NORMAL ECF NORMAL ICF
mOsm/L
80
0
Loss0.10 LIters
2 K2 Na
413
0
318
0
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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.
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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.
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Question 2: Which of the following test is the next step in the evaluation?
A. VCUG and urine culture
B. Renal sonogram and urine calcium to creatinine ratio
C. Urology referralD. CBC and Direct
CoombsE. Recheck in two
years
VCUG and urine cu
lture
Renal sonogram
and uri..
Urology r
eferral
CBC and Dire
ct Coombs
Recheck
in tw
o years
20% 20% 20%20%20%
6
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• 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.
More on Hematuria
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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
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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
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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.
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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 > 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.
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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
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March 17, 2005Trying to think about pediatric kidney disease - nephWHAT
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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).
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Question 3: The most likely cause of the gross hematuria is:
A. Myoglobinuria
B. Urinary tract infection
C. Obstructive uropathy
D. IgA nephropathy
E. Benign familial hematuria M
yoglobinuria
Urinary tr
act infecti
on
Obstructi
ve uro
pathy
IgA nephropath
y
Benign fa
milial h
ematuria
20% 20% 20%20%20%
6
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IgA
• IGA nephropathy– Boys > 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 > 10 yrs of age,
proteinuria, reduced GFR, hypertension and no macrohematuria
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Acute Glomerulonephritis
Low Complement Normal Complement
Systemic diseasesSLE
Subacute Bact EndocarditisShunt nephritis
Essential mixed cryoglobulinemiaVisceral abscess
Systemic diseasesPolyarteritis nodsa
Hypersensitivity vasculitisWegener’s
HSPGoodpasture’s
Renal diseasesAcute proliferative GN
Membranoproliferative GN
Renal diseasesIGA
RPGN -Anti-GBM, immune complex GN
Serologic evidence of antecedent strep infection(ASO, anti-Dnase B, streptozyme
PSAGNStrep endocarditis
LupusEssential mixed cryoglobulinemia
Shunt nephritisVisceral abscess
MPGNNon-strep infection
Clinical evidence to support endocarditis
Blood culturesEchocardiogram Treat PSAGN
Positive Negative
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Glomerular Non-glomerular
Urinalysis Dysmorphic RBCCellular castsBrown/tea colorBright redClotsCrystalsProtein
++
+++--+
--+
++++-
History Family Hx of ESRDSystemic diseaseNephrolithiasisTraumaSymptomatic vomiting
++---
--+++
Physical HypertensionSystemic signsEdemaAbdominal massGenital bruising
++++--
+--++
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Red or Tea colored/ Brown Urine
Fresh Centrifuged Urine Sample
Sediment Red withRed Cells
Supernatant Red withoutRed Cells
Hematuria
NOTE: If there is no red sediment, no RBCsand a clear supernatant, consider other causes such as urates, bile pigments,beets, porphyria, some medications, etc.
Hemoglobinuria*
Myoglobinuria
* Hemoglobinuria will havea red or pink hue to the serum
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On routine physical examination, an 8-year-old boy is found to have microscopic hematuria. The first step in your evaluation should be.
A. Examine the urine sedimentB. Order an renal ultrasoundC. Obtain a voiding cystourethrogramD. Perform a CBC in the officeE. Order an ASO titer and C3
Question 4
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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.
A. Complement studiesB. Serum creatinineC. Urinalysis for proteinD. Monitor blood pressure and urine outputE. Obtain a renal ultrasound
Question 5
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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
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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)
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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
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SO you
call this
urinary
frequency
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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.
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Question 6: Which of the following is the best approach?
A. Obtain a 1st AM urine for protein
B. Perform a complete biochemical profile
C. Obtain a C3, ASO and ANA
D. Refer for a renal biopsy
E. Schedule a renal sonogram and VCUG
Obtain a 1st
AM urin
e fo...
Perform
a complete bi...
Obtain a C3, A
SO and ANA
Refer for a
renal b
iopsy
Schedule a re
nal sonog...
20% 20% 20%20%20%
6
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What does Orthostatic Proteinuria mean?
Normal Orthostatic Recumbant
Erect
Threshold of Detection
ProteinExcretion
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• 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.
More on Proteinuria
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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
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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. UrinalysisB. Blood tests for total protein and albuminC. Serum creatinineD. Sedimentation rateE. Serum complement (C3)
Question 7
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March 17, 2005
Definitions (Pearl)
• Urine protein to creatinine ratio– Normal: < 0.2 (< 0.15 adolescents)– Mild to moderate: 0.2 to 1.0– Heavy or severe: > 1.0
• Persistent proteinuria: present both in the recumbent and the upright posture; even in this situation, proteinuira is less during recumbency
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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
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Nephrotic Syndrome - RXCorticosteroid 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.
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Hypertension
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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 > 99%ile for age and a height at ~50th %ile.
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Question 8: What is the most appropriate initial testing for this child?
A. Renal mag-3 flow scan
B. Electrolytes, BUN, Creatinine, Bicarbonate
C. Renal Sonogram with doppler
D. Urinary screening for drugs
E. 24 hour urine for catecholamines
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BP ClassificationGrade of hypertension Definition Appropriate next step
“White-coat” hypertension BP levels >95th 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 < 90th %ile
Pre-hypertension >120/80 mm Hg should be considered pre-hypertensive or90-95%ile
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 >5 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 >5 mm Hg higher than the 95th percentile, along with clinical signs or symptoms
Hospitalization and treatment to lower the blood pressure
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Estimate of Hypertension
Estimate without height adjustment1. If systolic BP equals or exceeds
100 + 2 times pt age in yrs
2. If diastolic BP equals or exceeds 70 + pt age in yrs
Estimate with height adjustment1. 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 heightAdd 2 mmHg to the value at the 50th %tile
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Historical Information
Physical Examination
Neonatal historyFamily historyDietary history
Risk Factors (smoking, alcohol use, drug use)
Non-specific / specific symtomatologyReview of Systems - sleep and exercise
patterns, etc.
Vital signs(including extremities)
Height/WeightSpecific attention to organ systems -
cardiac, eye, abdominal or other bruits, etc.
Consider ambulatory blood pressure monitor
Evaluation Phase 1
CBC, urinalysis, urine culture, electrolytes, BUN, creatinine, thyroid studies, uric acid
plasma renin, lipid profile, echocardiogram, renal ultrasound with duplex doppler
Evaluation Phase 2 Selected studies based on magnitude of the hypertension and/
or other clinical /laboratory findings
Renal flow scan (MAG 3)CT Angiography (CTA)
MRA (may not provide adequate evaluation for peripheral renal vascular lesions)
Renal arteriography with renal vein samplingPlasma / urine catecholamines and/or steroid concentrations
Evaluation of Hypertension
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Indications for Treatment
• Symptomatic hypertension
• Secondary hypertension
• Hypertensive target-organ damage
• Diabetes (types 1 and 2)
• Persistent hypertension despite nonpharmacologic measures
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Pharmacologic Therapy for Childhood Hypertension
• The goal for antihypertensive treatment in children should be reduction of BP to <95th percentile, unless concurrent conditions are present. In that case, BP should be lowered to <90th percentile.
• Severe, symptomatic hypertension should be treated with intravenous antihypertensive drugs.
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Urinary Tract Infections
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Case 9
• A 4 mo old girl presents with low grade fever, mid-lower abdominal pain and nighttime-incontinence. 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.
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Question 9: What is the most likely bacterial cause of her urinary tract infection?
A. Proteus mirabilis
B. E. coli
C. Coagulase positive Staphlococus
D. Alpha hemolytic Streptococcus
E. Klebsiella pneumoniae
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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%
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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
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Diagnosis
• Leukocyte test and nitrite test
• Urine culture > 40-50,000 CFU/mL
• Pyuria - not on recurrent UTIs
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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
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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
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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
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Grades of Reflux
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Reflux Recommendations“the simple way”
• GRADES I - III Antibiotics
• GRADES IV - V Surgery
• Although endoscopic approach is gaining favor over open reimplantation
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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)
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Feld - 10/98 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 > 100,000 colonies of E. Coli. Antibiotics are given.
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Question 10: What is the most appropriate next step?
A. Perform a DMSA renal scan
B. Refer to urology for cystoscopy
C. Perform a renal sonogram and VCUG
D. Perform urodynamics and flow studies
E. Repeat urine culture in 3 months
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Glomerulonephritis / Acute renal failure
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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.
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Question 11: What is the most likely diagnosis?
A. Henoch Schoenlein Purpura
B. Post streptococcal glomerulonephritis
C. IgA nephropathy
D. Acute pyelonephritis
E. Hemolytic uremic syndrome
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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
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HUS: Clinical manifestations
• Thrombocytopenia
• Hemolytic anemia
• Renal failure
• Neurologic (irritability, seizure, CVA)
• Pancreatitis (IDDM) and colitis
• Hypertension
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March 17, 2005
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HUS: Pathogenesis
• Endothelial cell damage occurs secondary to toxin injury via binding to glycolipid receptor or lipopolysaccharide absorption.
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HUS: Differential diagnosis
• Other forms of acute Glomerulonephritis / renal failure
• Vasculitis
• Urosepsis
• Renal vein thrombosis
• Coagulopathy (DIC)
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Conservative management
• Fluid restriction to <insensible losses plus urine output
• Foley catheter – limit to 24-48 hrs
• Blood transfusion / platelets
• Routine use of antibiotics controversial
• Diuretics
• Nutrition
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Surgical Complications
• Toxic megacolon
• Rectal prolapse
• Colonic gangrene
• Intussusceptions
• Perforation
• Strictures
• Mimic appendicitis, IBD
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BONUS 1John 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?
1.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.
2.Creatine supplementation affects hydration status and heat tolerance during exercise leading to muscle cramping and diarrhea
3.Creatine supplementation does improve physiological response to resistance exercise, increasing the maximal force production of muscles in men, not women.
4.Pasteurized cow's milk contains lower levels of creatine than human milk
5.Creatine has been shown to improve cognitive performance in young athletes.
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BONUS 2A 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:
1.Post Strep AGN
2.IgA nephropathy
3.Membranoproliferative glomerulonephritis
4.Membranous nephropathy
5.Wegener’s granulomatosis
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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 (Potter’s – 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
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• 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 > 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
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• 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 – 0157:H7 – MOST COMMON CAUSE OF
ACUTE RENAL FAILURE; low platelet count and microangiopathic hemolytic anemia (schistocytes; coombs negative)– cause undercooked meat or unpasteurized milk
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• 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.
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• 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 < 5.5– Serum bicarbonate usually less than 18– Remember FANCONI syndrome is RTA = glycosuria,
phosphaturia, and amino aciduria.– Treated with bicarbonate
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– Distal or Type I – impaired distal acidification; pH > 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
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• 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
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March 17, 2005
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Outline – Part 2
• Acute renal failure
• Chronic renal failure
• More Fluids & Electrolytes
• Tubular disorders
• Cystic kidney disease
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SCENARIOA 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?
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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
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ARF: DiagnosisPre-renal AGN ATN Obstruction
UA Marginal value
Key
RBC casts
RTEC Marginal value
SG >1.020 >1.020 1.008-1.012
1.008-1.012
UNa <20 <20 >40 >40
FENA <1% <1% >1% >1%
Uosm >400 >400 200-400 200-400
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March 17, 2005
ARF: Diagnosis
• AGN– PSAGN– HSP– SLE– MPGN– Wegener’s
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ARF: Diagnosis
• ATN– Unreversed pre-renal azotemia– Nephrotoxic meds– Contrast agents– High calcium, uric acid, phosphate– Rhabdomyolysis (myoglobin)– Intravascular hemolysis (hemoglobin)
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ARF: Diagnosis
• Obstructive uropathy– PUV– Prune belly– Vesicoureteric reflux– Neurogenic bladder (myelomeningocele)– Megacystis/megaureter– Secondary: stones, fibrosis
• Effect of age and gender
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ARF: Testing
• Key labs: BUN, creatinine, K
• EKG
• CXRay
• Renal ultrasound
• Specific blood tests based on underlying condition
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ARF: Management
• Urgent issues– Potassium
• Calcium• Glucose/insulin• NOT bicarbonate
– Blood pressure: parenteral therapy• Labetalol• Nitroprusside
– ECF volume
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ARF: Conservative Management
• Potassium– Diet restriction– Kayexalate
• Blood pressure– IV/PO meds
• ECF volume– Na restriction– Diuretic use – need for furosemide
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ARF: Indications for Dialysis
• Refractory hyperkalemia
• Refractory hypertension
• Symptomatic ECF volume overload
• Symptomatic azotemia– Infection– Bleeding– CNS changes
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ARF: Pearls
• Pre-renal azotemia and AGN are similar
• ATN and post-renal failure are similar
• Potassium kills first in ARF
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SCENARIOA 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 child’s problems?What are the likely causes his condition?How should his condition be treated?
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CKD: Diagnosis
• Stages– CKD I: renal injury GFR >90– CKD II: GFR 60-90– CKD III: GFR 30-60– CKD IV:GFR 15-30– CKD V: ESRD
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CKD: Common features
• Impact on growth
• Impact on bone: osteodystrophy
• Impact on puberty
• Impact on development – social and cognitive
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CKD: Causes
• Non-glomerular– Hypoplasia/dysplasia– Reflux nephropathy– Obstructive uropathy
• PUV• Prune Belly• Neurogenic bladder
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CKD: Clinical manifestations• Growth failure
– Dependent on age of onset– Dependent on level of GFR
• UTIs– Pyelonephritis
• Electrolyte abnormalities– Pseudohypoaldosteronism– Nephrogenic DI
• Neurocognitive disability
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CKD: Diagnosis
• Structural assessment
• Imaging studies– US– VCUG: dye vs radioisotope– DMSA scan– Retrograde studies, etc
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CKD: Diagnosis
ARFYounger child, abd mass, UTI
UAWBC, impaired concentration
US, VCUG, DMSA
Retrograde studiesCystoscopy, urodynamics
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SCENARIOA 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 adolescent’s problem?What are the most likely causes?How should her condition be treated?
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CKD: Causes
• Glomerular– FSGS– HUS– SLE– Membranoproliferative MPGN)– Alport– IgA Nephropathy– Membranous nephropathy– NOT diabetic or hypertensive nephropathy
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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
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CKD: Diagnosis
• Low value of radiology tests
• Blood tests– C3, C4, CH50– ASLO– ANA, dsDNA, Ro, La, Sm– ANCA– Anti-GBM– Renal biopsy
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CRF: Management
• Nutritional supplementations– CHO deficiency
• Protein restriction– Impact on growth– Effect in more advanced CKD
• BP control– Disease progression– ACEI/ARB
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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
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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
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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
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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
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SCENARIOA 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 38°C, 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/dlWhite cell count 30,000Platelets 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)?
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March 17, 2005
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
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Electrolyte disorders: Sodium
Assess ECF
High ECF Normal ECF Low ECF
Measure serum Na
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March 17, 2005
Electrolyte Disorders: Sodium
• History
• Source of Na loss
• Change in body weight
• Renal response to low ECGF volume– Oliguria– Reduced urine Na+– Reduced FENA
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Electrolyte disorders: Sodium
0
20
40
60
80
100
120
Normal Hypo Hyper
ICF
ECF
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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
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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
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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
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SCENARIOA 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 37°C, 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/dlWhite cell count 30,000Platelets 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)?
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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
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Electrolyte disorders: Potassium
• Regulatory organs– Kidney secretion
• Na+• Urine flow rate
– Adrenal• Aldosterone
– GI tract
• Transmembrane– pH– Osmolality– Beta adrenergics– Insulin
• Diet
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Potassium
• Key tests– BUN, Cr, Na, K, bicarbonate – Urine K useless– Urine Na/K ratio– Hormones
• PRA• Aldosterone
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Electrolyte disorders: Potassium
• Hyperkalemia– EKG
• Peaked T waves
– Treatment• Calcium infusion• Glucose/insulin• NOT Bicarbonate• Kayexalate• DIALYSIS
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Hyperkalemia: differential diagnosis
• No real disease– Increase cells: WBC, polycythemia, thrombocytosis,
crush injury– Transmembrane
• Renal– ARF– CRF– Liddle’s
• Adrenal– Adrenal failure– Congenital adrenal hyperplasia – ambiguous genitalia– Isolated renin abnormalities
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Hyperkalemia: Work-up
• BUN, creatinine, Na, K, Bicarbonate
• PRA
• Aldosterone
• Urinary Na/K ratio
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Electrolyte disorders: Potassium
• Hypokalemia– EKG
• U waves
– Treatment• Restore ECF volume to 2hyperaldosteronism• PO potassium
– Limitations: tolerance
• IV potassium– Limitation: 0.3 meq/kg/hr– Central vs peripheral IV
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Hyperkalemia: differential diagnosis
• Systemic– Malnutrition
• Adrenal– Adrenal overactivity– Congenital adrenal hyperplasia– Primary renin abnormalities
• Renal– DKA– Osmotic diuresis
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SCENARIOA 15 month child presents with a history of poor feeding and impaired growth. Prenatal and family history is non-contributory. On examination the infant’s 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 138Cl 114Bicarbonate 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)?
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Electrolyte disorders: acid-base
Acid load
AcuteChronic-Kidney
ProximalChronic-Kidney
Distal
RegenerateTitrated bicarbonate
Reclaim filtered bicarbonate
Lung
Large frequent doses
1-3 mmol/kg/day
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March 17, 2005
Electrolyte disorders
• Anion gap
• [Na] – {[Cl] + [HCO3]}
• Normal value: 4-12
• Impact of serum albumin
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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
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Electrolyte disturbances: RTA
• Proximal– Low K – Primary– Secondary
• Glycogen storage• Wilson’s, fructose intolerance, tyrosinemiaPTH, Vitamin D• Cystinosis
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Electrolyte disturbances: RTA
• Distal– Primary – Secondary
• Transplant rejection• Drugs: amphotericin, cisplatinum• Collagen vascular disease
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Electrolyte disorders: RTA
• Assessment– SMAC: Cl-– VBG: Bicarbonate– Urine: calcium, citrate– Urine anion gap: unmeasured cation (NH4+)– Xrays
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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
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Electrolyte disorders: Fanconi’s
Fanconi’sSyndrome
Complete proximal tubule dysfunction
RTA GlycosuriaPhosphaturia
TRPAmino Aciduria
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Electrolyte disorders: metabolic alkalosis
• Extrarenal/GI loss of K– CF
• Vomiting– NG suction– Pyloric stenosis
• Distal GI loss of bicarbonate– Chloride diarrhea
• Renal– Bartter’s– Gitelman’s– Apparent mineralocorticoid excess (AME)/licorice
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March 17, 2005
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
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Electrolyte disorders: DI
• Central– AVP replacement
• Nephrogenic– Adequate water intake– Low solute diet– Hydrochlorothiazide
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Electrolytes: Pearls
There are three pure renal causes of FTT – azotemia, DI, and RTA
RTA causes hyperchloremic acidosis
Bartter’s and Gitelman’s differ in calcium excretion – high in former low in latter
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Thank you
GOOD LUCK