Nephrology/Urology Flashcards
What is the main indication for orchidopexy?
When should a child be referred for orchidopexy?
When should the orchidopexy be performed?
Risk of infertility
Referral between 6-9 months
Between 6-18 months
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?
- 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
What is the best test to confirm pelvic urethral valves?
VCUG
What is an indication for a nuclear cystogram?
What is a disadvantage of a nuclear cystogram for assessment of first presentation UTI?
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.
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?
DMSA
MAG3 diuretic
Provide information on differential renal function
What are the 2 most common forms of DSD?
When should DSD investigations be initiated in an infant with hypospadias?
CAH, mixed gonadal dysgenesis
Bilateral cryptorchidism
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?
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
List 4 of the most common causes of high grade or “significant” congenital hydronephrosis
- UPJO: Ureteropelvic junction obstruction
- high-grade VUR: High-grade Vesicoureteral reflux
- UVJO: Ureterovesical junction obstruction
- PUV: Posterior urethral valves
What diagnosis is suspected when significant hydronephrosis is observed WITHOUT hydroureter?
UPJO - ureteropelvic junction obstruction
List 3 signs of PUV from antenatal ultrasounds?
What management would you recommend upon delivery of an infant with suspected PUV?
- 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
What is the most common cause of acquired hydronephrosis?
UPJO - ureteropelvic junction
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?
Idiopathic overactive bladder
Bacterial cystitis/Bladder outlet obstruction
Constipation, Nocturnal enuresis, recurrent cystitis
Provide 4 recommendations for the management of Idopathic Overactive Bladder
- Treat constipation if present
- Treat UTI if present
- Timed voiding (q1.5-2h)
- Observation if not bothersome to child
- Anticholinergics (oxybutynin)
- decrease frequency and intensity of involuntary contractions, resulting in increased bladder capacity
- Can worsen constipation → aggravate OAB
Which CAKUT anomaly is associated with:
Increased risk UVJO + VUR
Increased risk UPJO
Complete duplication
Horseshoe kidney
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?
CT KUB (2nd line though, shoudl get US first) - indicated when US identifies hydroephrosis/hydroureter but no stone identified
- Analgesic (NSAIDS +/- opioids)
- 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
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?
Calcium oxalate
- Increase hydration to 2-2.5L/day
- Ensure maintaining RDA intake for calcium
- Low sodium diet (associated with obligatory calcium excretion)
- Add citrus juice to water (contains citrate → urinary inhibitor of stone formation)
- 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)
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?
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
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?
Indirect hernia
If not resolved by 12 months
Reactive (infection, inflammation, trauma, tumour)
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
- Meatal stenosis
- Apply petroleum jelly to the glans for up to 6mo following circumcision
Acute complications:
- Minor bleeding
- Local infection
- Unsatisfactory cosmetic result
Medical indications
- Scarred phimosis (pathological phimosis)
- Recurrent balanoposthitis
- Genital lichen sclerosis (balanitis xerotica obliterans)
- Recurrent UTIs in high-risk patients as an adjunct or alternative to prophylactic antibiotics
- Untreatable paraphimosis
Contraindications
- Known bleeding disorder
- 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)
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?
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%
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?
- 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
- Nitrofurantoin - no longer commercially available as suspension; can crush, mix with yogourt or apple sauce
- TMP/SMX
Antibiotic resistance identified (even when believed to be contaminated). If resistance present to both Nitrofurantoin + TMP/SMX, consider discontinuing prophylaxis.
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?
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)
Provide counselling to a family who is considering use of bed alarms for primary nocturnal enuresis.
- 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
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?
- 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
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?
- 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
- Escherichia coli
- Klebsiella pneumoniae
- Enterobacter species
- Citrobacter species
- Serratia species
Adolescent females: Staphylococcus saphrophyticus
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?
- 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
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?
- 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
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.
-
Antenatal/Infant
-
Congenital Mesoblastic Nephroma
- treated with complete surgical excision
-
Congenital Mesoblastic Nephroma
-
>1 year
- Wilms tumour
- renal biopsy not done = “spillage” which upstages the tumour
- Wilms tumour
Wilms tumour syndromes:
- WAGR Syndrome
- Denys Drash syndrome
List 5 causes of painless scrotal swelling.
List 5 causes of painful scrotal swelling.
PAINLESS
- Testicular tumour
- Hydrocele
- Varicocele
- Large epididymal cyst
- Inguinal hernia
PAINFUL
- Orchitis
- Testicular torsion
- Incarcerated inguinal hernia
- Infectious epididymitis
- Torsion of the appendix testis
- Testicular tumour
How do you calculate insensible losses?
400mL/m^2/24h
What tests would you require in order to evaluate the cause of severe hyponatremia (sodium 120)?
- Urine sodium
- Serum osmolality
- Urine osmolality
List 4 causes of non-anion gap metabolic acidosis
USED CARP
- Ureteric diversion
- Sigmoid fistula
- Excess saline
- Diarrhea (or resolving DKA)
- Carbonic anhydrase inhibitors
- Addison’s
- Renal tubular acidosis
- Pancreatic fistula
List 4 causes of metabolic acidosis with an elevated anion-gap
MUDPILES
- Methanol toxicity
- Uremia
- Diabetic ketoacidosis
- Paraldehyde
- Isopropyl alcohol
- Lactic acidosis
- Ethylene glycol toxicity
- Salicylate toxicity
How do you find an RTA using urine electrolytes?
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)
What are the different types of RTA, their locations and features?
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
List 4 causes of Fanconi syndrome.
What are 4 expected features of laboratory investigations associated with Fanconi syndrome.
- Cystinosis
- Wilsons disease
- Galactosemia
- Tyrosinemia
- Herediatary fructose intolerance
- Lowe syndrome
- Mitochondrial disease
Lab features
- Non-anion gap metabolic acidosis
- Hypokalemia
- Hypophosphatemia
- Glucosuria
- Hyperphosphaturia
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.
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
- Dietary education (DASH diet)
- Increased physical activity
Pharmacologic therapy indications
- Symptomatic
- Target organ damage
- Stage 1 hypertension persisting ≥ 6 months, despite trial of non-pharmacologic therapy
- Stage 2 hypertension
- T1DM/T2DM, CKD or heart failure AND BP ≥90th
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?
- 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
Which antihypertensives can you start as first line?
When do you refer to pediatric nephrology?
What are the target BPs for management?
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
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?
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
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?
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
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
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
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?
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.
List 3 management strategies for management of nephritis.
What is the expected prognosis for PSGN?
- Fluid restriction
- Sodium restriction
- Treat hyperkalemia (if present) with low-potassium diet and kayexalate
- Close monitoring of fluid balance, daily weights, blood pressure
- 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%
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?
IgA Nephropathy + Alport syndrome
ACEi/ARB
Alport syndrome
- Progressive sensorineural hearing loss (SNHL)
- Lens/retina anomalies
- Esophageal/tracheobronchial leiomyomas
What 3 features are seen with prune belly syndrome?
What is the most common non-urologic manifestation seen with this diagnosis?
- Bilateral cryptorchidism
- Abdominal wall laxiety from deficient musculature
- Urinary tract dilation (bilateral hydroureteronephrosis)
Pulmonary hypoplasia
List 3 risk factors for renal vein thrombosis
- Prematurity
- Maternal gestational diabetes or preeclampsia
- Perinatal asphyxia
- Hypercoagulability (factor V leiden, protein C deficiency)
- Polycythemia
- Sepsis
- UVC malplacement
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?
- Thrombocytopenia
- Hemolytic anemia
- Renal insufficiency
Shiga-toxin-producing Escherichia coli (STEC)
- Significant proteinuria persisting 1 year after episode
List 4 reasons for normocytic anemia in children with chronic renal failure.
- Lack of erythropoeitin production
- Poor nutrition secondary to anorexia and vomiting due to uremia - low iron, folate, B12
- Hemodialysis causing blood loss
- Reduced red cell life span
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.
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)
What is the monitoring process for glomerular disease post-HSP?
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)
Name the ‘synpharyngitic’ nephritic syndromes?
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)
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) this value reflects the mothers creatinine.
What anomaly is most likely to be found when there is a single umbilical artery: d. Renal anomaly e. No anomaly f. Cardiac anomaly
d. Renal anomaly
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
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
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
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)
What are some key differentiating features between multi cystic dysplastic kidney disease and polycystic kidney disease?
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)
In a newborn found to have a multicystic-dysplastic kidney, the following is likely: a. hematuria b. hypertension c. ureteropelvic reflux
c. ureteropelvic reflux
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
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
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) 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
What’s the most common cause of abdo mass in newborn? a) hydronephrosis b) polycystic kidneys c) neuroblastoma
a) hydronephrosis - hydronephrosis and multi cystic dysplastic kidneys present with abdo masses in newborns (NOT polycystic kidneys)