Nephrology/Urology Flashcards

1
Q

What is the main indication for orchidopexy?

When should a child be referred for orchidopexy?

When should the orchidopexy be performed?

A

Risk of infertility

Referral between 6-9 months

Between 6-18 months

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2
Q

What are the criteria for the TWIST score? (SHARE)

What management is indicated based on the scoring?

When should a testicular torsion be repaired by?

A
  • Swelling of testicles - 2
  • Hard testes - 2
  • Absent cremasteric reflex - 1
  • Riding high testicle - 1
  • Emesis/nausea - 1

Indications

  • 0-2 LOW risk → no ultrasound or consult required
  • 3-4 INTERMEDIATE risk → ultrasound warranged
  • ≥5 HIGH risk → ultrasound not required, urgent urological consultation and surgery required

Repair - ideally within 6h of onset of pain, if not available, manual detorsion can be attempted

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3
Q

What is the best test to confirm pelvic urethral valves?

A

VCUG

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4
Q

What is an indication for a nuclear cystogram?

What is a disadvantage of a nuclear cystogram for assessment of first presentation UTI?

A

Initial assessment for VUR in females only.

Follow-up of VUR in males and females

Unable to detect presence of posterior urethral valves in males.

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5
Q

Which nuclear diagnostic test is able to assess renal scarring?

Which nuclear diagnostic test is best able to asess obstruction?

Why are renal scans performed?

A

DMSA

MAG3 diuretic

Provide information on differential renal function

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6
Q

What are the 2 most common forms of DSD?

When should DSD investigations be initiated in an infant with hypospadias?

A

CAH, mixed gonadal dysgenesis

Bilateral cryptorchidism

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7
Q

Why shouldn’t RBUS be done within the first 2 days of life?

What are the recommendations for postnatal imaging in antenatal diagnosis of hydronephrosis?

What is the management recommendations with postnatal imaging results?

A

Can underestimate the severity of hydronephrosis.

  • In 3rd trimester, APD 7-10 = Low grade, >15 = High grade
    • Low grade (“minimal or mild”, calyces are not dilated)→ RBUS within first 1-3 months of life, may benefit from antibiotic prophylaxis
    • High grade (“moderately severe”, dilated calyces, parenchymal thinning) → RBUS within first 2 weeks of life

Management

  • If normal, counsel on signs and symptoms of UTI/UPJO and discharge
  • If APD <10 - repeat RBUS in 6 months, then annually. No antibiotic prophylaxis
  • If >15 - refer to pediatric urology for further investigations
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8
Q

List 4 of the most common causes of high grade or “significant” congenital hydronephrosis

A
  1. UPJO: Ureteropelvic junction obstruction
  2. high-grade VUR: High-grade Vesicoureteral reflux
  3. UVJO: Ureterovesical junction obstruction
  4. PUV: Posterior urethral valves
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9
Q

What diagnosis is suspected when significant hydronephrosis is observed WITHOUT hydroureter?

A

UPJO - ureteropelvic junction obstruction

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10
Q

List 3 signs of PUV from antenatal ultrasounds?

What management would you recommend upon delivery of an infant with suspected PUV?

A
  • Male sex suspected
  • Distended, thick walled bladder
  • Bilateral hydronephrosis
  • Keyhole sign
  • Oligohydramnios

Postnatal management

  • Urgent postnatal RBUS
  • Bladder decompression if needed
  • Confirmatory VCUG
  • Serial creatinine levels
  • Consult urology
  • Consider nephrology consult for acid-base, electrolyte management if needed
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11
Q

What is the most common cause of acquired hydronephrosis?

A

UPJO - ureteropelvic junction

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12
Q

What is the most common non-infectious cause of daytime incontinence?

What 2 diagnoses do you need to rule out before you can diagnose someone with OAB?

What are common associated diagnoses with OAB?

A

Idiopathic overactive bladder

Bacterial cystitis/Bladder outlet obstruction

Constipation, Nocturnal enuresis, recurrent cystitis

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13
Q

Provide 4 recommendations for the management of Idopathic Overactive Bladder

A
  1. Treat constipation if present
  2. Treat UTI if present
  3. Timed voiding (q1.5-2h)
  4. Observation if not bothersome to child
  5. Anticholinergics (oxybutynin)
    • decrease frequency and intensity of involuntary contractions, resulting in increased bladder capacity
    • Can worsen constipation → aggravate OAB
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14
Q

Which CAKUT anomaly is associated with:

Increased risk UVJO + VUR

Increased risk UPJO

A

Complete duplication

Horseshoe kidney

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15
Q

What is the best investigation to most reliably identify a non-obstructing stone in the distal ureter?

List 2 management strategies in the ER for renal stones

When is urology referral indicated for kidney stones?

A

CT KUB (2nd line though, shoudl get US first) - indicated when US identifies hydroephrosis/hydroureter but no stone identified

  1. Analgesic (NSAIDS +/- opioids)
  2. Alpha-adrenergic blockers (tamsulosin) to hasten passage of small ureteric stones

Referral

  • Too large to pass (>5mm)
  • Unremitting pain
  • Persistent, severe obstruction
  • Solitary kidney
  • Infected, obstructed kidney
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16
Q

What is the most common type of renal stone?

List 5 dietary recommendations for kidney stones

What type of investigations should you complete for a renal stone?

A

Calcium oxalate

  1. Increase hydration to 2-2.5L/day
  2. Ensure maintaining RDA intake for calcium
  3. Low sodium diet (associated with obligatory calcium excretion)
  4. Add citrus juice to water (contains citrate → urinary inhibitor of stone formation)
  5. Low protein diet

Investigations

  • Initial
    • Lytes, BUN, Creatinine
    • Urinalysis with stone evaluation
      • pH <6 → uric acid stones
      • pH >7 → calcium phosphate and struvite stones
  • Additional
    • Renal stone analysis (if retrieved)
    • Urine Ca:Cr ratio
    • 24-hour urine sample to assess for risk factors of stone development (cystinuria, hyperuricosuria, hypocitraturia, hyperoxaluria, hypercalciuria)
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17
Q

What is the most common metabolic abnormality associated with stone formation?

List 4 risk factors for calcium stone development

What bacterial cystitis is associated with struvite stones?

What amino acids are increased with cystine stones?

A

Hypercalciuria

Risk factors

  • Hypercalciuria
  • Hypercalcemia
  • Immobilization
  • Loop diuretics
  • Ketogenic diet
  • Renal diseases (distal RTA [RTA1], medullary sponge kidney)
  • Intestinal malabsorption (IBD, CF, celiac disease [oxalate])

Proteus mirabilis

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18
Q

What type of hernia is at risk of developing with a communicating hydrocele?

When should you refer for hydrocele?

What are reasons for a persisting non-communicating hydrocele?

A

Indirect hernia

If not resolved by 12 months

Reactive (infection, inflammation, trauma, tumour)

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19
Q

In regards to circumcision:

What is the most common long-term complication + how can it be prevented?

List 2 acute complications

List 2 medical indications

List 2 contraindications

List 3 possible benefits

A
  • Meatal stenosis
  • Apply petroleum jelly to the glans for up to 6mo following circumcision

Acute complications:

  1. Minor bleeding
  2. Local infection
  3. Unsatisfactory cosmetic result

Medical indications

  1. Scarred phimosis (pathological phimosis)
  2. Recurrent balanoposthitis
  3. Genital lichen sclerosis (balanitis xerotica obliterans)
  4. Recurrent UTIs in high-risk patients as an adjunct or alternative to prophylactic antibiotics
  5. Untreatable paraphimosis

Contraindications

  1. Known bleeding disorder
  2. Hypospadias

Benefits

  • ↓incidence UTI in young boys
  • ↓risk of penile cancer
  • ↓risk of trichomonas, BV and cervical cancer in female partners
  • ↓risk of acquiring STI (HSV, HIV, HPV)
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20
Q

What percentage of males will have retractile foreskins by 6yo + 17yo?

What is the difference between paraphimosis and phimosis?

What is the treatment of phimosis?

A

50% + 95%

  • Phimosis = scarred, thickened foreskin preventing retraction
  • Paraphimosis = foreskin entrapped behind the glans

Treatment

  • Topical steroid BID to foreskin, accompanied with gentle traction
    • Betamethasone 0.05%
    • Triamcinolone 0.1%
    • Mometasone furoate 0.1%
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21
Q

When does the CPS recommend antibiotic prophylaxis be considered?

What is the length of time prophylaxis should be considered?

What are 2 usual choices for prophylaxis?

When should prophylaxis be stopped or changed?

A
  • Grade IV-V VUR
  • Significant urological anomaly

Consider managing constipation appropriate to decrease UTI recurrences.

Length of time: No more than 3-6 months

Usual choices - 1/4-1/3 of treatment dose daily

  1. Nitrofurantoin - no longer commercially available as suspension; can crush, mix with yogourt or apple sauce
  2. TMP/SMX

Antibiotic resistance identified (even when believed to be contaminated). If resistance present to both Nitrofurantoin + TMP/SMX, consider discontinuing prophylaxis.

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22
Q

What is secondary nocturnal enuresis?

When should a child be considered enuric?

How common is bedwetting in 5yo, 8yo and 15yo?

What sleep disorder is associated with nocturnal enuresis?

What treatment options are available?

A

Bladder control attained for ≥6 months, then incontinence reoccurs

If wetting >2/week when >5yo

  • 5yo - 10-15%
  • 8yo - 6-8%
  • 15yo - 1-2%

Sleep terrors

Management

  • Minimize emotional impact on the child
    • Reassurance, support and avoidance of punishment and humiliation → maintain self-esteem + minimize parental frustrations
  • Non-pharmacological:
    • Avoid caffeine-containing foods
    • Avoid excessive fluids before bedtime
    • Take child out of diapers
    • Include child in morning cleanup in nonpunitive manner
    • Ensure access to toilet
  • Behavioural therapy
    • “Dry bed training” may be more effective in combination with alarm therapy
    • Ensure to empty bladder before bed
    • Avoid drinking fluids after supper, if able
    • Encourage the child to participate in cleaning up in morning
  • If “significant problem for child”
    • Alarm devices - most efficacious
    • DDAVP/desmopressin acetate for camp/sleepovers or if alarm system impratical
      • Avoid fluids 1h before + 8h after taking
    • Imipramine
      • Short-term treatment
      • Refractory cases with distressed, older children
      • Parents are reliable + counselled about safe storage (danger of overdose)
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23
Q

Provide counselling to a family who is considering use of bed alarms for primary nocturnal enuresis.

A
  • Success depends on motivation of child and willingness of child/parents/siblings to be awakened
  • Most effective ≥7-8yo
  • Cure rate is just under 50%
  • Recommend a trial period of 3-4 months. It can take 1-2mo to see any effect
    • Usually first improvement is less urine than totally dry
  • Discontinue once 14 days consecutively dry
    • Can consider “overlearning” at this point - where you encourage child to drink 2 glasses water before bed, then discontinue once 7 days dry in a row
  • May be repeated if a relapse occurs
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24
Q

When should UA/UCx be obtained from children <3yo?

What is the most common cause of a positive urine culture in afebrile young children?

List 4 symptoms of UTI in children ≥3yo

What 5 features can be used to rule out UTI in girls <24 months? How is it used?

A
  • Fever >39˚C with no apparent source

Contamination

Symptoms

  • Increased Urinary frequency
  • Dysuria
  • Hematuria
  • Abdominal pain
  • Back pain
  • New daytime incontinence

Predictive tool (females <2yo)

If ≤1 present, risk for UTI is <1%

  • Age <12mo
  • Fever >39˚C
  • White race
  • Fever for >2 days
  • Absence of another source of infection
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25
Q

What can cause a nitrite test to be falsely negative?

What is considered pyuria?

Who requires a catheterized or SPA collection for urine culture to be considered reliable?

What are 4 of the most common causes of UTI in previously well children who have not been on antibiotics?

Which bacteria is seen with adolescent females with UTI?

A
  • If bladder is emptied frequently
  • Infection 2˚ organism that does not metabolize nitrate (all gram positive organisms)

Pyuria = >5 WBC/hpf

Children not toilet trained

Common causes

  1. Escherichia coli
  2. Klebsiella pneumoniae
  3. Enterobacter species
  4. Citrobacter species
  5. Serratia species

Adolescent females: Staphylococcus saphrophyticus

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26
Q

What is the minimum colony count indicative of UTI for clean catch and catheter urines?

When should blood cultures be performed in a child with UTI?

When should renal function be monitored?

List 2 features of a complicated UTI

What route of antibiotic is recommended?

A
  • Clean catch: ≥10^5 CFU/mL or ≥10^8 CFU/L
  • Catheter: ≥5 x 10^4 CFU/mL or ≥5x10^7 CFU/L

Blood cultures: HD unstable

Renal function: >48h aminoglycosides or complicated UTI

Complicated UTI (get RBUS to r/o abscess or obstruction)

  • Hemodynamically unstable
  • No clinical improvement within 24h or fever not trending downward within 48h of appropriate antibiotics
  • Bladder or abdominal mass
  • Elevated serum creatinine (at any time)
  • Poor urine flow

Route of antibiotic:

  • Uncomplicated Febrile UTI:
    • PO if can RECEIVE and TOLERATE every dose
    • 2-3mo old: PO w/close follow-up arranged (others feel IV needed)
  • Complicated UTI: IV
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27
Q

What is the duration of treatment recommended for:

  • Uncomplicated UTI?
  • Cystitis (dysuria/urinary frequency + NO FEVER)
  • Complicated UTI

What should be done if multiresistant drug isolated?

What imaging studies should be performed, why and when?

What counselling should you provide to families?

A
  • Uncomplicated UTI: 7-10 days
  • Cystitis: 2-4 days
  • Complicated UTI:

Multiresistant organism isolated:

  • Asymptomatic: Repeat UA/UCx and change therapy only if results are suggestive of persistent UTI
  • Symptomatic: Repeat UA/UCx and modify antimicrobial based on results

Imaging studies

  • RBUS
    • Who: First febrile UTI AND <2yo
    • When: within 2 weeks of acute illness
    • Why:
      • Identify if severe VUR or structural anomalies present
      • Confirm child had pyelonephritis
    • Results:
      • If significantly abnormal or VUR IV-V → consult paediatric urology or nephrology to determine whether urgent need for consult is needed and make plan for further investigation/management
  • VCUG: consider for males <2yo + 2nd well-documented UTI
  • Nuclear cystogram: consider for females <2yo + 2nd well-documented UTI

Counselling

  • Advise parents their child needs to be assessed for possibility of recurrent UTI early in the course of any unexplained fever
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28
Q

What is the most common “solid” renal mass diagnosed at the following times?:

Antenatal/Infant

>1 year old

List 2 syndromes associated with Wilms tumour.

A
  • Antenatal/Infant
    • Congenital Mesoblastic Nephroma
      • treated with complete surgical excision
  • >1 year
    • ​Wilms tumour
      • renal biopsy not done = “spillage” which upstages the tumour

Wilms tumour syndromes:

  1. WAGR Syndrome
  2. Denys Drash syndrome
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29
Q

List 5 causes of painless scrotal swelling.

List 5 causes of painful scrotal swelling.

A

PAINLESS

  1. Testicular tumour
  2. Hydrocele
  3. Varicocele
  4. Large epididymal cyst
  5. Inguinal hernia

PAINFUL

  1. Orchitis
  2. Testicular torsion
  3. Incarcerated inguinal hernia
  4. Infectious epididymitis
  5. Torsion of the appendix testis
  6. Testicular tumour
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30
Q

How do you calculate insensible losses?

A

400mL/m^2/24h

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31
Q

What tests would you require in order to evaluate the cause of severe hyponatremia (sodium 120)?

A
  • Urine sodium
  • Serum osmolality
  • Urine osmolality
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32
Q

List 4 causes of non-anion gap metabolic acidosis

A

USED CARP

  • Ureteric diversion
  • Sigmoid fistula
  • Excess saline
  • Diarrhea (or resolving DKA)
  • Carbonic anhydrase inhibitors
  • Addison’s
  • Renal tubular acidosis
  • Pancreatic fistula
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33
Q

List 4 causes of metabolic acidosis with an elevated anion-gap

A

MUDPILES

  • Methanol toxicity
  • Uremia
  • Diabetic ketoacidosis
  • Paraldehyde
  • Isopropyl alcohol
  • Lactic acidosis
  • Ethylene glycol toxicity
  • Salicylate toxicity
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34
Q

How do you find an RTA using urine electrolytes?

A

Urinary anion gap = Na+K-Cl

Cannot be interpreted if UNa <25.

if Na+K > Cl = Ammonia excretion

If Na+K < Cl = Normal (No RTA)

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35
Q

What are the different types of RTA, their locations and features?

A

Proximal (Type 2) + Fanconi syndrome

  • impaired resorption of bicarb/glucose/protein/etc
  • proximal tubule
  • acidic urine
  • Tx: PO4 supp and vit D

Type 4

  • aldosterone deficiency/insensitivity (can’t excrete K)
  • collecting duct
  • Hyperkalemia
  • Tx: kayexelate

Distal (Type 1)

  • impaired excretion of acid (H+)
  • distal tubule
  • basic urine + hypokalemia + hypercalcuria
  • Tx: thiazides
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36
Q

List 4 causes of Fanconi syndrome.

What are 4 expected features of laboratory investigations associated with Fanconi syndrome.

A
  1. Cystinosis
  2. Wilsons disease
  3. Galactosemia
  4. Tyrosinemia
  5. Herediatary fructose intolerance
  6. Lowe syndrome
  7. Mitochondrial disease

Lab features

  1. Non-anion gap metabolic acidosis
  2. Hypokalemia
  3. Hypophosphatemia
  4. Glucosuria
  5. Hyperphosphaturia
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37
Q

List 3 risk factors for hypertension

What is the criteria for diagnosis of HTN in children?

List 2 non-pharmalogical recommendations for HTN management.

List 3 indications for initiation of pharmacologic therapy.

A

Risk factors for HTN

  • History of coarctation of the aorta or aortic arch obstruction
  • Diabetes mellitus
  • Renal disease
  • Obesity

Criteria for HTN Diagnosis

  • if sBP or dBP ≥95th on ≥3 occasions (AAP says then ABPM if Stage 1 over 3 visits or elevated BP for ≥1y)
    • If stage 1: ≥2 occasions within 1 month
    • If stage 2: prompt referral for evaluation and therapy

Non-pharmacological management

  1. Dietary education (DASH diet)
  2. Increased physical activity

Pharmacologic therapy indications

  1. Symptomatic
  2. Target organ damage
  3. Stage 1 hypertension persisting ≥ 6 months, despite trial of non-pharmacologic therapy
  4. Stage 2 hypertension
  5. T1DM/T2DM, CKD or heart failure AND BP ≥90th
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38
Q

What are the stages of hypertension as per AAP?

What investigations should be performed after HTN has been diagnosed as per CHEP?

What additional investigations are recommended by AAP?

A
  • Normal <90th
  • Elevated BP ≥90 or 120/<80-129/<80
  • Stage 1 HTN ≥95 or 130/80-139/89 (whichever is lower)
  • Stage 2 HTN ≥95th +12mmHg or ≥140/90 (whichever is lower)

Investigations (CHEP)

  • Screening
    • Electrolytes, BUN/Creatinine, total CO2
    • Renal ultrasound with dopplers
    • Urinalysis
  • Cardiovascular risk screen
    • Fasting blood glucose
    • Lipid profile
  • End-organ damage assessment
    • Retinal examination
    • Echocardiogram
    • Albumin-to-creatinine ratio (1st morning)

AAP Investigations

  • Renal ultrasound if <6yo or abnormal UA/renal function tests
  • If obese
    • HbA1C
    • AST/ALT - fatty liver disease screen
  • Depending on history/physical + initial testing
    • CBC - growth delay or abnormal renal function
    • Sleep study - if OSA risk factors
    • Drug screen
    • TSH
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39
Q

Which antihypertensives can you start as first line?

When do you refer to pediatric nephrology?

What are the target BPs for management?

A

1st line:

  • ACEi (not in black patients)
  • ARB
  • long-acting dihydropyridine CCB

Referral to nephro: ≥6mo standard-dose monotherapy without improvement

Target BPs:

  • No RFs <95th
  • RFs or target organ damage <90th
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40
Q

When should competitive sport participation be restricted in HTN?

In severe symptomatic hypertension

  • list 3 medications you can use and their routes
  • What principle do you need to follow when lowering the BP?
A

Sport restriction for:

  • Stage 2 HTN (lower below threshold before participating)
  • Presence of LVH or concomitant heart disease

Acute Severe HTN

  • Life threatening
    • Esmolol - IV infusion
    • Hydralazine - IV/IM
    • Labetalol - IV infusion or bolus
    • Nicardipine - IV infusion or bolus
    • Sodium Nitroprusside - IV infusion
  • Less significant symptoms
    • Clonidine - PO
    • Hydralazine - PO

Principle: reduce by no more than 25% over first 8h

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41
Q

What are the 2 most common causes of proteinuria?

List 2 etiologies of false positive proteinuria on urinalysis

How would you recommend testing a child who has elevated protein on initial assessment?

A

Orthostatic proteinuria, Transient proteinuria

False positive

  • ​Alkaline urine
  • Concentrated urine, gross hematuria
  • Chlorhexidine contamination
  • Radioconstrast agent (iodine)

Investigations (if 24h urine, protein <1g/24h)

  • 1st morning urine x 2 at least 1 week apart
    • Urinalysis with microscopy
    • Urine protein/creatinine ratio
      • If <0.2, no further testing needed
      • If >0.2 → Refer to nephrology
        • CBC, lytes, BUN/Cr, lipid profile, C3, C4 +/- ANA (esp. if FMHx)
        • Consider RBUS
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42
Q

Regarding nephrotic syndrome:

What is the most common cause? What is its prognosis?

List 3 features that it is defined by.

List 4 management strategies.

List 2 complications associated with it

A

Minimal change nephrotic syndrome (MCNS) - 95% respond to prednisone, most attain long-term remission

Nephrotic Syndrome: (>3.5g/24h)

  • Hyperlipidemia
  • Hypoalbuminemia
  • Edema
  • Proteinuria

Management

  • 1st line: Glucocorticoids
  • Low-salt diet
  • Furosemide +/- albumin (if severe edema)
  • If relapsing/steroid-dependent:
    • Cyclophosphamide
    • Cyclosporine or tacrolimus

Complications

  • Infections - risk for pneumococcal infections
    • Spontaneous bacterial peritonitis
    • Cellulitis
  • Venous thrombosis
    • DVT
    • Cerebral venous thrombosis
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43
Q

Regarding glomerulonephritis:

What is the most common cause?

List 2 diagnoses associated with normal C3.

List 2 diagnoses with decreased C3 and C4.

If PSGN was caused by a skin infection, what is the best test to confirm systemic effect?

A

Poststreptococcal glomerulonephritis (PSGN)

Normal C3

  • Alport syndrome
  • IgA nephropathy
  • ANCA vasculitides
  • HSP nephritis

Low C3/Low C4

  • Membranoproliferative glomerulonephritis (MPGN)
  • Lupus nephritis
  • Postinfectious glomerulonephritis
  • Shunt nephritis

DNAse B (elevated with PSGN skin/throat) but ASOT will be normal in skin infections.

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44
Q

List 3 management strategies for management of nephritis.

What is the expected prognosis for PSGN?

A
  1. Fluid restriction
  2. Sodium restriction
  3. Treat hyperkalemia (if present) with low-potassium diet and kayexalate
  4. Close monitoring of fluid balance, daily weights, blood pressure
  5. If hypertensive without improvement following restriction:
    • 1st line: Diuretics - HCTZ or furosemide
    • CCB and beta-blockers

Prognosis

  • Proteinuria should resolve in ~4wks, C3 normalize in ~8wks
  • Microhematuria can persist for a “few years”
  • Long-term:
    • Persistent hematuria/proteinuria in 5-20%
    • Chronic kidney disease 1%
    • Hypertension 3%
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45
Q

What 2 diagnoses should you consider with URTI and painless, gross hematuria

In chronic nephritic syndrome, what medication is typically considered for proteinuria >0.5g/day?

What features are associated with Alport syndrome?

A

IgA Nephropathy + Alport syndrome

ACEi/ARB

Alport syndrome

  • Progressive sensorineural hearing loss (SNHL)
  • Lens/retina anomalies
  • Esophageal/tracheobronchial leiomyomas
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46
Q

What 3 features are seen with prune belly syndrome?

What is the most common non-urologic manifestation seen with this diagnosis?

A
  • Bilateral cryptorchidism
  • Abdominal wall laxiety from deficient musculature
  • Urinary tract dilation (bilateral hydroureteronephrosis)

Pulmonary hypoplasia

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47
Q

List 3 risk factors for renal vein thrombosis

A
  1. Prematurity
  2. Maternal gestational diabetes or preeclampsia
  3. Perinatal asphyxia
  4. Hypercoagulability (factor V leiden, protein C deficiency)
  5. Polycythemia
  6. Sepsis
  7. UVC malplacement
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48
Q

What are the 3 main features of hemolytic uremic syndrome?

What is the most common cause of HUS?

What one clinical feature would make you think you had to follow their renal function over the long term?

A
  1. Thrombocytopenia
  2. Hemolytic anemia
  3. Renal insufficiency

Shiga-toxin-producing Escherichia coli (STEC)

  • Significant proteinuria persisting 1 year after episode
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49
Q

List 4 reasons for normocytic anemia in children with chronic renal failure.

A
  1. Lack of erythropoeitin production
  2. Poor nutrition secondary to anorexia and vomiting due to uremia - low iron, folate, B12
  3. Hemodialysis causing blood loss
  4. Reduced red cell life span
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50
Q

You are present at the delivery of a 30wk premature baby. The medical student asks you how much sodium to put into the IV solution.

What would you tell her?

What are 2 reasons for your choice.

A

None.

  • Lack of sodium promotes postnatal diuresis and acute contraction of extracellular compartment
  • Renal capacity to retain and excrete sodium load is reduced (would lead to hypernatremia + respiratory distress if given)
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51
Q

What is the monitoring process for glomerular disease post-HSP?

A

Late-onset nephritis may develop up to 6 months after acute illness:

  • Checked UA and BP weekly for the first 4 weeks, then monthly for 6 months
  • Cases with severe renal involvement may need empiric immune suppression or even renal transplant (1-2% develop ESRD)
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52
Q

Name the ‘synpharyngitic’ nephritic syndromes?

A

Synpharyngitic: within 1-2 days of pharyngitis

  • IgA nephropathy
  • Alport Syndrome
  • MPGN

Note: PSGN is NOT synpharyngitic (usually appears after 1-2 weeks post pharyngitis or 3-6 weeks post skin infection)

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53
Q

A newborn infant has Cr of 83. Which of these statements is true? a) this value reflects the mothers creatinine. b) this is a normal value for a newborn infant.

A

a) this value reflects the mothers creatinine.

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54
Q

What anomaly is most likely to be found when there is a single umbilical artery: d. Renal anomaly e. No anomaly f. Cardiac anomaly

A

d. Renal anomaly

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55
Q

5 day old infant in nursery has low urine output. Creatinine is 120. Renal ultrasound shows absent left kidney. The right kidney is at the low end of normal for size, and has consistent/uniform echodensity throughout. Which of the following is most likely? a) Prognosis depends on liver and lung status b) Likely will progress to end stage renal disease in childhood c) Likely will progress to end stage renal disease in mid adulthood d) he will have normal renal function

A

b) Likely will progress to end stage renal disease in childhood (Yes! Kidney should compensate so a normal or small is not normal as it should be hypertrophied. + Cr high + Kidney not uniform consistancy normally so wondering about dysplasia or hypoplasia. Without a good sole kidney can progress to RF) - lung usually not affected because the single kidney is able to compensate - ideally, if have unilateral renal agenesis the other kidney compensates - creatinine should be normal and the other kidney should be hypertrophied

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56
Q

What is true of a patient with multicystic dysplastic kidney disease? a. chance of VUR b. likely that first degree relative will be affected c. usually causes hematuria d. early hypertension

A

ANSWER: a. chance of VUR b. likely that first degree relative will be affected (not usually inherited vs. PCKD is) c. usually causes hematuria (false; AR PCKD can) d. early hypertension (rare to have HTN; usually late)

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57
Q

What are some key differentiating features between multi cystic dysplastic kidney disease and polycystic kidney disease?

A

MCDK: kidney replaced by cysts - no normal tissue; can have ureteral atresia/contralateral VUR; not inherited, most common cause of abdo mass in newborn; unilateral PCKD: AD (most common - large cysts develop over time) or AR, can be associated with syndromes like TS, von Hippel Lindau, Bardet-Biedl; bilateral; other organs affected (especially hepatobiliary)

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58
Q

In a newborn found to have a multicystic-dysplastic kidney, the following is likely: a. hematuria b. hypertension c. ureteropelvic reflux

A

c. ureteropelvic reflux

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59
Q

A newborn infant has a left sided abdominal mass. A renal ultrasound demonstrates multi cystic kidney disease. Which of following would this be associated with: a) ipsilateral hydroureter b) posterior urethral valves c) sensorineural hearing loss d) cataracts e) risk of malignancy

A

ANSWER: e) risk of malignancy (yes - wilms tumour arising from tissue even if cysts regress) - hydroureter likely contralateral - PUV not an association - SNHL and cataracts not an association

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60
Q

6-year-old girl with incidental finding of a 2 cm renal cyst. Appropriate management: a) observe and repeat US b) CT abdomen c) ultrasound liver d) urology consult e) full nephrologic workup

A

a) observe and repeat US Re: liver U/S - hepatbiliary issues more related to AR PCKD - simple cysts with normal renal function only need observation - complex cysts may have risk of cancer - need more ix

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61
Q

What’s the most common cause of abdo mass in newborn? a) hydronephrosis b) polycystic kidneys c) neuroblastoma

A

a) hydronephrosis - hydronephrosis and multi cystic dysplastic kidneys present with abdo masses in newborns (NOT polycystic kidneys)

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62
Q

Baby with weak abdominal musculature. Cryptorchidism. Baby in intubated in NICU. Bilateral abdominal masses on exam. What is this associated with. a) Bilateral wilms b) Polycystic kidneys c) Multicystic kidneys d) Hydronephrosis

A

d) Hydronephrosis - description of Prune Belly syndrome (aka triad or Eagle Barrett syndrome) - urinary tract abnormalities from urethral obstruction as fetus - massive hydroureter and hydronephrosis, large bladder, patent urachus, VUR - oligohydramnios, pulmonary hypoplasia, malrotation

63
Q

Midline mass and E.coli urosepsis, next diagnostic step: a) VCUG b) renal U/S

A

b) renal U/S - renal U/S is initial screen for first febrile UTI in child less than 2 - mass suspicious for hydronephrosis

64
Q

Newborn with increased creatinine, palpable mass in midline. How do you confirm the diagnosis? a. VCUG b. Abdo Ultrasound c. CT abdo

A

b. Abdo Ultrasound - concern is about an obstructive renal lesion - 2/3 of abdominal masses in neonates are renal in origin - boys with PUV have walnut size mass above pubic symphysis

65
Q

You are referred an otherwise healthy 16 year old boy from his family doctor after proteinuria was noted on a routine urine dipstick. What are 2 reasons for a false positive for protein on dipstick? What is the most common reason for persistent proteinuria?

A
  1. false positives: - high urine pH (>7) - highly concentrated urine specimen - contamination of the urine with blood 2. most common cause of persistent proteinuria: postural proteinuria (found in 60% of kids with persistent proteinuria) persistent proteinuria) - have proteinuria when upright, but normal urine protein when supine - NOT associated with hematuria, hypertension, hypoalbuminemia, edema or renal dysfunction
66
Q

What test do you do to confirm postural/orthostatic proteinuria?

A

diagnosis: obtain first morning urine sample and test for urinalysis and urine protein:cr ratio - correct method of getting this sample: fully empty bladder before bed at night, then collect sample from first void immediately upon getting out of bed in the morning - obtain sample for 3 consecutive days

67
Q

15 yo female with 2+ protein on routine exam. What to do? a) 24 hr urine protein b) first morning urine analysis x 2 c) Renal function tests

A

b) first morning urine analysis x 2

68
Q

A child has progressive periorbital and peripheral edema, abd pain and distention for 1 week. She is now febrile with a temperature of 39.2 degrees. Blood pressure is within normal limits and her abdomen is diffusely tender. On U/A there is no blood but there is 4+ protein. What is the most likely diagnosis: 1. Post-strep GN 2. Nephritic syndrome with peritonitis 3. HUS 4. Appendicitis

A
  1. Nephritic syndrome with peritonitis (?nephrotic syndrome more likely - maybe typo?)
69
Q

Pt had GAS 2 weeks ago. Now presents with hemoglobin 70, Platelets of 30 and rising Cr and BUN. What is the diagnosis? a) HUS b) HSP

A

a) HUS - HSP should not have anemia or TCP - patients with familial HUS can be triggered by preceding illness

70
Q

Decreased C3 is a feature of which of the following: a. IgA nephropathy b. HUS c. post strep glomerulonephritis d. nephrotic syndrome

A

c. post strep glomerulonephritis - other causes of low C3: membranoproliferative GN, SLE - kidney issues with normal C3: IgA nephropathy, idiopathic rapidly progressive GN, anti-GBM disease, HSP, Goodpasture’s, Alport, granulomatosis with polyangiitis (Wegner’s)

71
Q

Which of the following is associated with a low C3: a) Alport’s syndrome b) nephrotic syndrome c ) post-streptococcal glomerulonephritis d) hemolytic uremic syndrome e) Henoch-Schonlein purpura

A

c ) post-streptococcal glomerulonephritis (YES)

72
Q

All of the following about Alport’s are true except: 1. Girls have worse prognosis 2. Bad prognosis is with gross hematuria in childhood 3. Progressive sensorineural hearing loss in childhood 4. 15% have end stage renal disease before age 15 5. 2-3% of all end stage renal disease is due to Alport’s

A
  1. Girls have worse prognosis - Alport = hereditary nephritis - all have micro hematuria, may have gross hematuria, may have proteinuria - progressive bilateral SNHL - poor prognosis: gross hematuria in childhood, nephrotic syndrome, prominent GBM thickening, male - mgmt: ACEi slows rate of progression
73
Q

A child with HSP will have: a. increased IgA (yes! If done) b. decreased Hb and platelets c. decreased immunoglobulins

A

a. increased IgA - most common childhood vasculitis - IgA deposition causing abdo pain (FOBT+, intussusception), arthritis, renal (microscopic hematuria/proteinuria)

74
Q

Pt with HSP. What would you do to monitor: a. urinalysis b. IgA c. Stool for o/b d. AXR

A

a. urinalysis - weekly while active disease, then mostly x6 months - also monitor BP

75
Q

Child had a URTI a week ago. He now presents with bloody diarrhea, abdominal pain and a petechial rash. What is his diagnosis?

A

HSP

76
Q

An 8 year old boy presents with hematuria and hypertension. His ASOT is positive. List one test that you could do that would support your diagnosis of post-infectious glomerulonephritis.

A
  • complement C3 level (reduced in >90% of cases of post strep GN in the acute phase, returns to normal within 6-8 weeks post infection) - PSGN occurs following staph, strep, gram negative bacterial infections, flu, parvo
77
Q

Post strep GN. What two lab tests that would confirm your dx of PSGN.

A
  • complement C3 (expect to be low) - ASOT for proof of recent strep infection
78
Q

Periorbital swelling with no tenderness, no fever and normal blood pressure. What do you do? a. reassure b. check for proteinuria c. start antibiotics

A

b. check for proteinuria - edema is the most common presenting symptom in children with nephrotic syndrome

79
Q

Child with nephrotic syndrome treated with steroids, weaned off 3 months ago and was clinically well. Now presents with albumin 10, ascites, 3+ protein in urine. Name three specific therapies

A

mgmt of relapsed nephrotic syndrome: - repeat course of prednisone - acute mgmt: Na restriction (<1500mg daily), diuresis (furosemide - loop diuretic), fluid restriction if hyponatremia - low fat diet if dyslipidemia

80
Q

What are the diagnostic findings for minimal change nephrotic syndrome (most common type)?

A
  • periorbital edema progressing to generalized - urine protein:cr ratio >2 - lytes, BUN, Cr usually normal - hypoalbuminemia - high cholesterol and triglycerides
81
Q

When should secondary nephrotic syndrome be suspected and how do you investigate?

A
  • gross hematuria, HTN, renal insufficiency, age <1 or >12 - C3 (normal in MCD, low in other causes), ANA, dsDNA, Hep B, Hep C, HIV; kidney biopsy in kids over 12
82
Q

A child with nephrotic syndrome has recently been started on a course of oral steroids. Which of the following vaccines is contraindicated? A) Prevnar B) Hep B C) Influenza D) Varicella zoster

A

ANSWER: D) Varicella zoster - live attenuated vaccine is contraindicated in patients with immunocompromise including high dose steroids A) Prevnar - inactivated bacterial B) Hep B - Recombinant Viral C) Influenza - nasal spray would be contraindicated (live attenuated viral vaccine), but all injected flu vaccines are inactivated or recombinant and would not be contraindicated

83
Q

You are assessing a 3 week old infant. The weight is 4 kg, the birth weight was 3.6 kg, the blood pressure is 90/55. The mother states that the infant is feeding well. The labs show: Na 142 K 3.6 Cl 113 Cr normal pH 7.25, urine pH 5.0 What is the most likely etiology a. Hyperaldosteronism b. RTA, proximal c. CF d. Psychosocial failure to thrive

A

b. RTA, proximal RTA: normal AG metabolic acidosis (less HCO3 reabsorbed - proximal, or less H+ out - distal) - type 1: distal - HCO3 <15, urine pH >5.5, hyperCauria - type 2: proximal - HCO3 >15, urine pH <5.5 - type 4: hypoaldosteronism - high K, low Na *acidic pH means distal tubule is working fine - so not type 1 or 4

84
Q

Which of the following will be found in a 6 year old with a distal RTA: a. Glycosuria b. Hypercalciuria c. Metabolic alkalosis

A

b. Hypercalciuria

85
Q

Child presents with failure to thrive, polydipsia, polyuria, and hypokalemic metabolic alkalosis. a) hyperaldosteronism b) Bartter syndrome c) cystinosis d) renal tubular acidosis e) congenital adrenal hyperplasia

A

ANSWER: b) Bartter syndrome - hypoK, metabolic alkalosis, hypercalciuria, salt wasting - dysmorphic: triangle face, protruding ears, large eyes, droopy mouth a) hyperaldosteronism (K low but not polyuria/dipsia) c) cystinosis (polyuria/dipsia, low K BUT non AG metabolic acidosis) e) congenital adrenal hyperplasia ( not enough aldosterone = high K+, low Na)

86
Q

How do you differentiate Bartter from Gittelman syndrome?

A

Both metabolic alkalosis and hypoK, but Gittelman has hypocalciurua and low magnesium

87
Q

A 3-month-old boy born at term is failing to thrive. He is otherwise asymptomatic. Labs show Na 142, K 6.5, Cl 114, Cr 45, HCO 3 14, pH 7.24. a) renal failure b) renal tubular acidosis c) hyperaldosteronism d) cystic fibrosis e) Fanconi syndrome

A

b) renal tubular acidosis

88
Q

14 mos male, FTT, vx, met acidosis, pH 7.31, bicarb 14, K 3.5, Na140, Cl 118, urine pH 6.3 a distal RTA b Bartters c organic acidopaty d nutrit. deprivation

A

a distal RTA metabolic acidosis with normal K and alkalotic urine= poor H into urine= distal issue= type 1 - Bartters - alkalosis

89
Q

The following are shared by cystinosis and renal tubular acidosis EXCEPT: a) hypokalemia b) nephrolithiasis c) concentrating defect d) aminoaciduria e) hyperchloremic metabolic acidosis

A

d) aminoaciduria Features of cystinosis: - french Cdn, fair complexion and blonde hair - healthy at birth - develop FTT, polyuria, polydipsia, dehydration - photophobia by 3-6 years - nephrocalcinosis

90
Q

Infantile cystinosis. What do you get? a. cataracts b. end stage renal failure c. nephrocalcinosis

A

b. end stage renal failure

91
Q

Infant post cardiac surgery with poor urine output. BUN 25, Cr 177, urine (low Na, concentrated) a. ATN b. prerenal failure c. anaesthetic injury to kidney d. obstructive uropathy e. renal vein thrombosis

A

b. prerenal failure (likely related to CO; low urine Na means body trying to keep in Na due to perceived low intravascular volume) - Patients whose urine shows an elevated specific gravity (>1.020), elevated urine osmolality (UOsm > 500 mOsm/kg), low urine sodium (UNa < 20 mEq/L), and fractional excretion of sodium (FENa) <1% (<2.5% in neonates) most likely have prerenal ARF.

92
Q

What are some lab features that differentiate renal from prerenal causes of AKI?

A

● Intrinsic AKI: specific gravity < 1.010, urine osmolality low (<350 mOsm/kg), high urine Na (>40), fractional excretion > 2% (> 10% in neonates)= likely intrinsic ● Pre-renal AKI: elevated specific gravity (> 1.0200), elevated urine osmolality (> 500), low urine Na (< 20), fractional excretion < 1% (< 2.5 for neonates)

93
Q

15 yo post trauma observed for 24 hr and d/c home. Presents a day later with orange urine and oliguria and high Cr, no improvement with fluids a) rhabdomyolysis b) Renal vein Thrombosis c) Pre renal failure

A

a) rhabdomyolysis

94
Q

A 17 year old boy presents to the ED 48 hours after being in an MVC with oliguria and orange coloured urine. There was a prolonged extrication from the car. Despite adequate hydration in hospital his creatinine rises from 57 to 177. What is the next step in your management: a. Epinephrine perfusion b. Norepinephrine perfusion c. Mannitol perfusion

A

c. Mannitol perfusion (theoretical benefit; pull fluid into vasculature and improve urinary flow)

95
Q

A 10 year old boy presents to the ED post-MVC with significant fractures and a BP of 50/30. He is resuscitated, but over the course of 48 hours in hospital his creatinine rises and he begins to have polyuria with a U/O of 5L/d. What is the most likely cause of his condition: a. Fat embolus b. ATN c. DM

A

b. ATN

96
Q

A 12 month old has had diarrhea for 3 days and low-grade fever. One stool had scant blood. His urine output has decreased since yesterday. On examination he is alert and interactive, has a HR of 150, BP 82/60, and skin turgor is increased. What is the most appropriate initial management: a. IV rehydration b. PO rehydration

A

b. PO rehydration

97
Q

Description of child with renal failure. What are four mechanisms for his anemia?

A
  • decreased erythropoietin production (predominant cause) - iron deficiency from chronic blood loss (frequent phlebotomy, surgery, dialysis) - B12 deficiency - folate deficiency - hyperparathyroidism
98
Q

Toddler with gastroenteritis and moderate dehydration. What management is most appropriate: a) give IV fluids now b) give IV fluids if vomiting c) give oral solution containing 20-65 mmol of Na d) stop breastfeeding and give electrolyte solution e) hold re-feeding for 72 hours

A

c) give oral solution containing 20-65 mmol of Na (AAP says 45-50 Na for mild-moderate dehydration; WHO says 75; European says 60) · Contraindications: protracted vomiting despite small frequent feeds, severe dehydration, paralytic ileus, monosaccharide malabsorption

99
Q

Best oral rehydration solution in a child with gastroenteritis and dehydration: a) sodium 20 mmol/L, potassium 20 mmol/L, glucose 10% b) sodium 60 mmol/L, potassium 20 mmol/L, glucose 2% c) sodium 60 mmol/L, potassium 0 mmol/L, glucose 8% d) ginger ale e) apple juice

A

b) sodium 60 mmol/L, potassium 20 mmol/L, glucose 2%

100
Q

Which of the following usually accompanies hypernatremic dehydration? a. hypercalcemia, hypoglycemia b. hypocalcemia, hyperglycemia c. hypercalcemia, hyperglycemia d. hypocalcemia, hypoglycemia

A

b. hypocalcemia, hyperglycemia - there is a Na-Ca exchanger - so if Na is high to pull water in, Ca is by definition lost

101
Q

8 month old baby with gastroentereitis. Na 160, Cl elevated, bicarb 14, HR 220, BP 60/30. What is your initial management. Once stable what is your ongoing fluid management?

A
  1. Bolus 20ml/kg 0.9% normal saline by IV over 20 minutes and repeat as necessary until BP and HR normalize 2. calculate fluid deficit based on degree of dehydration + maintenance fluid + ongoing losses (vomiting and diarrhea) - use D5/0.45%NS to start and monitor Na q2-4h - do not lower Na by more than 0.5mEq/h –> easier real life option and depending on how much space on exam: D545 +20KCl at 1.25-1.5x maintenance
102
Q

2 day old infant presents with fever of 39.5 ax. He is breast-fed and his weight has fallen from 3.8 to 3.5 kg. He is lethargic but rouses during the examination. His fontanelle is normal. Na 150, K 7.3, Cl 110, BUN 8, Cr 110, uncong bili 190, normal CBC. What is the likely diagnosis? a ) hypernatremic dehydration due to decreased fluid intake b) hypertonic breast milk c) diabetes insipidus d) sepsis e) meningitis

A

Hypernatremic + Hyperkalemia + Jaundice. a ) hypernatremic dehydration due to decreased fluid intake

103
Q

6 yr old previously well with 10 minute generalized tonic clonic seizure. Hypertensive , HR 90, Na 115 , Urine Na 30 . Euvolemic on exam. What is your initial management? List 2 possible etiologies.

A
  1. 3% NS 5ml/kg IV push 2. euvolemic hyponatremia: - SIADH, glucocorticoid deficiency (adrenal insufficiency), hypothyroid, water intoxication
104
Q

Child with nephrotic syndrome admitted with edema. Na=125. Best fluids: 1. D5W/0.2% saline 2. D5W/0.45% saline 3. normal saline 4. 3% saline 5. nothing

A
  1. nothing (usually water + Na restrict and if need to resus albumin + diuretics) Asymptomatic Hyponatremia Big Picture o Hypovolemic= restore deficit w/ isotonic fluid o Hypervolemic= water + Na restrict +/- diuretics (after albumin if low) o Euvolemic= eliminate excess water
105
Q

3 wk male infant vx, lethargy, poor po intake, Na 118, K8, most important lab test a vasopressin b 17 OHP c calcium d renin

A

b 17 OHP CAH

106
Q

5 year old with asthma. Treated with ventolin overnight and Q 30 this morning. Aminophyline added this morning to help improve oxygenation. Child complaining of nausea and weakness. You should check: A) serum sodium B) serum glucose C) serum potassium D) serum magnesium

A

C) serum potassium

107
Q

Which of the following is present in tumour lysis syndrome: a) hyperuricemia b) hyponatremia c) hypokalemia d) hypophosphatemia e) hypercalcemia

A

a) hyperuricemia

108
Q

Child with vomiting and diarrhea who was fed a home concoction of enteral feed. Now is lethargic and seizing. Na 115, creatinine elevated. Ur Na 12, Bicarb 18, glucose 3.5. Which is the best next step: 1. Administer 3% NaCl at a rate to increase Na by 2mEq/L/hr. 2. Give Lasix to increase urine output 3. Give bicarb 4. Give bolus of D5W0.45 20cc/kg

A
  1. Administer 3% NaCl at a rate to increase Na by 2mEq/L/hr.
109
Q

2 yo Kid with gastro now mod dehydration. VS normal. K+ 2.9, HCO3 12, pH 7.3. what next step? a. ORS 300cc/h x 4 h b. ORS 5 cc/hr x 24 h c. IVF D5 ½ NS d. IVF NS bolus

A

a. ORS 300cc/h x 4 h

110
Q

week old with pyloric stenosis, severe metabolic alkalosis (bicarbonate 34). What to do: 1. Give hydrochloric acid IV 2. OR immediately 3. Give large amounts of chloride IV 4. Give 5mmol/kg of KCl IV bolus 5. Give ascorbic acid IV

A
  1. Give large amounts of chloride IV
111
Q

Newborn with 6 mm pelvicaliectasis documented on antenatal U/S. What to do? 1. Prophylax with antibiotics 2. U/S prior to discharge 3. U/S in 3 weeks 4. VCUG

A
  1. U/S prior to discharge
112
Q

Antenatal U/S shows moderate hydro. U/S at 24 hrs normal. What to do next and why.

A
  • repeat AUS after the first 3 days of life (wait for a normal neonatal fluid balance/urine output) - measuring at 24 hours is too early and can lead to false negatives
113
Q

6mm pelicalviceal dilatation noted on antenatal and postnatal u/s. What should be done next? a) start prophylactic antibiotics b) VCUG c) DMSA d) repeat U/S in 2 months

A

d) repeat U/S in 2 months

114
Q

2-day old baby with antenatal hydro, confirmed on post-natal U/S to have moderate-severe hydronephrosis. BW shows normal renal function, baby seems to be peeing well. a. consult urology prior to discharge b. DMSA c. arrange for an outpatient VCUG d. follow up in 2 months

A

a. consult urology prior to discharge - advice regarding need for prophylaxis on discharge - moderate to severe hydronephrosis does also need VCUG for further investigation (timing of VCUG - inpatient or outpatient?)

115
Q

What is most common kidney stone in pediatrics? a) Ca oxylate c) Struvate d) Cystine e) Uric acid

A

a) Ca oxylate - (many causes - hypercalciuria, increased Ca absorption from gut, renal leak (decreased tubular reabsorption of calcium), primary hyperparathyroidism, iatrogenic, loop diuretics, ketogenic diet, corticosteroids, exogenous adrenocorticotropic hormone, theophylline, distal RTA type I (calcium phosphate stones), hypocitraturia (citrate inhibits calcium crystallization), high vitamin D, immobilization, sarcoid, cushing disease, heterozygous cystinuria, hyperoxaluria) c) Struvate - (magnesium ammonium phosphate - secondary to UTI, foreign body, urinary stasis) d) Cystine - secondary to cystinuria e) Uric acid - hyperuricosuria, Lesch-Nyhan syndrome, myeloproliferative disorders, post-chemo, IBD

116
Q

Teenager found to have struvite stone. What would be next investigation? a) Urine calcium/Po4 b) Urine culture c) 24 hour urine ca

A

b) Urine culture UTI caused by urea-splitting organisms (most often proteus, klebsiella, e. Coli, pseudomonas) result in excessive urinary alkalinization and excessive production of ammonia which can lead to precipitation of magnesium ammonium phosphate (struvite) and calcium phosphate

117
Q

Description of a child with renal stones. Was a prem. Which medication was the child likely on in the neonatal period that would contribute to this picture? a. gentamicin b. furosemide c thiazide

A

b. furosemide - nephrocalcinosis is associated with urinary stones. The most common causes are furosemide, distal RTA, hyperparathyroidims, medullary sponge kidney, hypophosphatemic rickets, sarcoidosis, hyperoxaluria, prolonged immobilization, cushing syndrome, hyperuricosuria, renal candidiasis

118
Q

An 8 year old female presents with microscopic hematuria. She has no symptoms. The most likely diagnosis is: a. Alport’s b. Wilms’ tumour c. IgA nephropathy d. Nephrocalcinosis e. post streptococcal glomerulonephritis

A

e. post streptococcal glomerulonephritis

119
Q

An 8-year-old boy has recurrent episodes of asymptomatic gross hematuria triggered by intercurrent illnesses. Between illnesses, dipstick shows microscopic hematuria. Best test to determine etiology: a ) immunoglobulins b) C3 and C4 levels c) 24-hour urine protein d) abdominal ultrasound e) DMSA scan

A

a ) immunoglobulins - if positive makes IgA nephropathy very likely, though note than 15% of the healthy population has high IgA

120
Q

5 yo male with fever and rhinorrhea presents with gross hematuria one day later. He has had a previous similar episode 3 months ago. What is the most likely diagnosis? a) chronic glomerulonephritis b) post-Strep GN c) postinfectious GN d) IgA nephropathy e) nephrotic syndrome

A

d) IgA nephropathy (yes; recurrent, short latency period of 1-2 days) b) post-Strep GN (no usually 7-21d latency and then last much longer)

121
Q

7 year old with microscopic hematuria most likely dx? a. IgA nephropathy b. alports c. idiopathic

A

a. IgA nephropathy

122
Q

PIGN vs. IgA - which test differentiates? a. immunoglobulins b. C3 c. 24-hour urine collection d. ANA

A

b. C3 - low in post-infectious GN, normal in IgA nephropathy

123
Q

Child with hematuria found during pre-camp check-up (incidental finding). What would you do next?

A
  1. repeat the urinalysis in a few days to see if spontaneously resolves. Do urinalysis with microscopy to confirm RBCs seen. - asymptomatic child with microscopic hematuria on 3 dips in a 2 week period should have: urine culture, urine ca:cr ratio +/- sickle screen. If normal, next step is urinalysis in all first degree relatives. Renal and bladder ultrasound then indicated to rule out tumour, cystic disease, hydronephrosis, urolithiasis. If normal, then check serum lytes and creatinine. - most likely if all Ix negative is benign microscopic hematuria (often runs in families)
124
Q

4 yo female with persistent microhematuria on 2 urine dips. Grandfather has renal stones. What investigation would you do to prove the diagnosis (1)

A

renal and bladder ultrasound to rule out stones

125
Q

A child has bright red urine a few days after getting a URTI. What is the diagnosis?

A

IgA nephropathy

126
Q

Most common cause hypertension in adolescents: a) primary (essential) hypertension b) renal artery stenosis c) congenital renal disease d) coarctation of the aorta e) pheochromocytoma

A

a) primary (essential) hypertension

127
Q

Which is a possible etiology of neonatal hypertension: a) autosomal recessive polycystic kidney disease b) multicystic kidney disease c) maternal eclampsia d) unilateral hydronephrosis e) peripheral pulmonic stenosis

A

a) autosomal recessive polycystic kidney disease

128
Q

A 6-year-old child has hypertension and persistent hypokalemia. Most likely diagnosis: a) hyperaldosteronism b) Bartter syndrome c) Addison’s disease d) renal vein thrombosis e) aortic coarctation

A

a) hyperaldosteronism c) Addison’s disease (primary adrenal insufficiency- not enough steroid hormones -> abdo pain, FTT, darkening of skin, adrenal crisis= low BP, LOC, high K and low Na)

129
Q

A 7 year old child has an acute onset of hematuria, but is systemically well with no history of illnesses or recent medication use. His HR is 110, RR is 24 and he is afebrile. His initial blood pressure is 140/87. What is your medication choice for initial management of his blood pressure: 1. IV nitroprusside 2. Salt and H2O restriction 3. SL nifedipine

A
  1. SL nifedipine
130
Q

Kid with hypertension confirmed by ambulatory blood pressure monitoring. Has BP 121/86. What do you do? a. start him on captopril b. observe and repeat an ambulatory blood pressure test c. renal ultrasound d. 24 h urine catecholamines

A

c. renal ultrasound - stage 1: BP 95-99th percentile plus 5mmHg - can evaluate prior to starting treatment - stage 2: BP >99th percentile plus 5mmHg - prompt evaluation and pharmacologic therapy

131
Q

14 year old girl with SLE. Hypertensive emergency BP 180/100. Name 3 drugs you would use to treat her hypertension and their route of administration

A

IV hydralazine, IV labetolol, IV sodium nitroprusside (if she truly has hypertensive emergency). Given her lupus and the high likelihood of lupus nephritis given the hypertension, ACE-inhibitors or angiotensin receptor blockers would be indicated for hypertension treatment as these also reduce proteinuria, but not for acute tx.

132
Q

15 year old with hypertensive emergency. List two medications that you can use to lower BP acutely. For each medication, list one side-effect (not including hypotension).

A
  1. hydralazine - palpitations 2. labetolol - bradycardia 3. nicardipine - nausea Basically all (if you need to guess): palpitations, nausea, SOB
133
Q

RVT etiology: a) UVC b) IDM with polycythemia and dehydration

A

b) IDM with polycythemia and dehydration - sudden onset gross hematuria, unilateral/bilateral flank masses, HTN, oliguria

134
Q

6 month M with UTI and found to have grade 2 reflux on one side. What to do? 1. Prophylax with amoxil 2. Prophylax with septra 3. Do Urine C+S monthly 4. Repeat VCUG in 6 months

A
  1. Repeat VCUG in 6 months (CPS: no prophylaxis unless gr. 4-5 or signif uro anomaly)
135
Q

6 yo female with prior history of recurrent UTI, 2 of them afebrile, known bilateral reflux. Last VCUG shows ongoing low grade reflux. She has been off antibiotics and infection free for 2 years. What to do next? a) Restart antibiotics b ) Observe only c) VCUG every 2 years

A

b ) Observe only

136
Q

3 month old boy with UTI and grade 2 VUR on Right side. Which is the most clinically relevant way to detect renal scarring. 1. IV pyelography 2. nuclear renal scan (DMSA) 3. Renal excretion scan (MAG-3 or DTPA) 4. VCUG

A
  1. nuclear renal scan (DMSA)
137
Q

5 mo baby with UTI. Give 2 indications for prophylaxis. Give 2 indications for referral to urology

A

prophylaxis: grade IV-V VUR, or unusually abnormal renal bladder ultrasound (anomaly of kidney or urinary tract) - per CPS statement referral to urology: same

138
Q

Child with back pain. Ultrasound shows left hydronephrosis. Test to give Dx? A. Mag-3-Scan 99mTc-DTPA for function b. Urine culture

A

A. Mag-3-Scan 99mTc-DTPA for function (renal scintigraphy assesses renal anatomy and function) - nuclear medicine test that provides images of the kidneys to help determine their level of functioning and if there are any obstructions. They can be very important in the diagnosis of hydronephrosis and UPJ obstructions (which present with abdo/flank or back pain).

139
Q

Photograph of Prune Belly Syndrome. Which of the following is a consistent association: a. Posterior urethral valves b. Progression to renal failure c. Sex-linked inheritance d. Polyhydramnios

A

b. Progression to renal failure (long term 1/3 can have end stage renal failure)

140
Q

You are seeing an otherwise healthy 4 month old boy for a routine visit in your office. On examination you note that he has an undescended testis on the right side a. At what age is referral to a Pediatric General Surgeon appropriate in the case of an undescended testis? b. Name 2 complications that can occur if an undescended testis is not repaired.

A

a. most descend in first 3 months; if not down by 4 months then unlikely to come down so refer *note testes descend at 7-8 months gestation so normal for prems to be undescended b. decreased fertility - poor testicular growth - malignancy (more difficult to screen)

141
Q

The reason that we do orchidopexy is: a) reduce chance of testicular cancer b) allow testicular examination c) increase fertility potential

A

c) increase fertility potential

142
Q

Description of testicular torsion. How can this diagnosis be confirmed? a) testicular ultrasound b) nuclear scan

A

a) testicular ultrasound

143
Q

Adolescent boy with nonpainful testicular mass. What test should be done? a) Ultrasound b) Mumps serology c) CT pelvis

A

a) Ultrasound

144
Q

Testicular torsion a) Blue discoloration of the scrotum is pathognomonic b) Absence of cremasteric reflex is common c) 36 hrs to treat before losing testis

A

b) Absence of cremasteric reflex is common *a) Blue discoloration of the scrotum is pathognomonic (blue dot in keeping with torsion of appendix testis)

145
Q

10 mo irritable. Acute onset of red scrotal swelling. Parents had noted on + off swelling in the past few days. Today irritable, VSS mild tachycardia. On exam Rt red swollen and transilluminates well. What is the diagnosis? b. Epididimytis c. Testicular torsion d. Acute hydrocele e. Incarcerated hernia

A

e. Incarcerated hernia

146
Q

Teenager presents with acute scrotal pain. A) List two important features on physical exam. B) List 4 differential diagnoses. C) What two things would you do for management?

A

A) - swelling - discolouration - **cremasteric reflex (absent in testicular torsion) - **abnormal lie of testis (e.g. higher than normal - seen in torsion) B) - testicular torsion - torsion of appendix testis - epididymitis - trauma - ruptures testis, hematocele - incarcerated inguinal hernia - mumps orchitis - testicular vasulitis C) - urology consult - ultrasound - pain medication - if suspicious of torsion can attempt manual detorsion

147
Q

Description of a baby with a reducible hernia. When should he be referred to surgery for repair?

A

immediately if inguinal as risk of incarceration is highest in infants - if umbilical hernia: surgery is not advised unless hernia persists to 4-5 years of age, causes symptoms, becomes strangulated or becomes progressively larger after age 1-2 years.

148
Q

Which of the following has the lowest relapse rate when used for primary enuresis in a 7 year old boy? a) Imipramine b) DDAVP c) alarm system d) nighttime fluid restriction

A

c) alarm system

149
Q

4 yr girl with post void enuresis. What is most likely diagnosis?

A

fused labia (incontinence occurs after voiding when girl stands up - run-off from behind labia)

150
Q

8 year old child is embarrassed by nocturnal enuresis. What percent persists to adulthood? 3 treatments?

A
    • 20% of children over age 5 have nocturnal enuresis; resolves spontaneously in 15% of children every year thereafter; <1% of adults continue to have nocturnal enuresis 2. reassurance - limit fluid intake to 60mls after 6pm - no caffeine after 4pm - motivational therapy (star chart) - bedwetting alarm - pharmacologic (second line in specific situations only) -DDAVP
151
Q

The most common cause of secondary enuresis in a 7 y/o boy is: 1. DM 2. Constipation 3. Psychiatric problem 4. UTI 5. Nephrogenic DI

A
  1. Constipation
152
Q

A seven year old boy presents with bedwetting. Which of the following is more common with a diagnosis of primary enuresis than with secondary enuresis: a. Family history b. Polydipsia

A

a. Family history

153
Q

What are the lab findings in SIADH?

A
  • Decreased urine output
  • Plasma Osm < 270
  • Urine Osm > Plasma Osm
  • low BUN
  • Urine Sodium > 20 MEq/L
154
Q

What is the ratio for compensation for resp acidosis/alkalosis?

A